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Rheumatology revison notes

The document provides detailed information on autoimmune diseases, particularly systemic lupus erythematosus (SLE), systemic sclerosis, and Sjögren’s syndrome. It discusses the clinical features, diagnostic criteria, laboratory findings, and treatment options for each disease, emphasizing the significance of specific antibodies and associated risk factors. Additionally, it describes manifestations affecting various organ systems and outlines prognostic indicators.

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11-03-2018 Rheumatology TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Homogeneous • Ab against ds DNA nucleosomes & histone Peripheral rim • Ab against ds DNA & to nuclear envelope protein TONY SCARIA 2010 KMC
11-03-2018 Speckled pattern • Ab against nuclear non DNA contents • Sm Ag  SLE • SSA & SSB sjogren syndrome • Ribonucleopattern  Mixed connective tissue ds Nucleolar pattern • Ab to RNA in systemic sclerosis Centromere pattern • Anticentromere Ab in systemic sclerosis TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 SLE TONY SCARIA 2010 KMC • Differential Diagnosis of Rash over face (Malar) • 1. Non erythematous- • 1. Chloasma • 2. Erythematous- • 1. SLE • 2. Photosensitivity reaction • 3. Contact dermatitis Sparing of nasolabial fold TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Renal involvement TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Wire loop lesion • Formed by subendothelial deposits • Seen in class 3,4 and 5 • Most commonly a/w class 4 • It indicates active ds & poor prognosis TONY SCARIA 2010 KMC Adverse prognosis • Factors associated with Adverse Prognosis and High Risk of Renal Progression in Lupus Nephritis • Hypertension • Hypocomplementemia • Thrombocytopenia • High Anti Ds DNA antibody titres • Pregnancy • Nephritic urinary sediment • Azotemia • Proteinuria • Decreased GFR TONY SCARIA 2010 KMC
11-03-2018 Better prognosis • Presence of anti LA (SSB) indicate lower involvement of kidney in SLE TONY SCARIA 2010 KMC • Most common cause of death in SLE with in 10 years is renal failure (followed by infection) • But most common over all cause is cardiac failure TONY SCARIA 2010 KMC
11-03-2018 Muskuloskeletal system • Mc system to be involved in SLE • Non erosive arthritis TONY SCARIA 2010 KMC CNS • Psychosis a/w anti P Ab (antiribosomal P Ab) TONY SCARIA 2010 KMC
11-03-2018 Lungs involvement in SLE • Pleuritis and pleural effusions (the most common pulmonary manifestations). • Alveolar injury (edema and hemorrhage) • Chronic interstitial fibrosis. • Shrinking lung syndrome • The chest radiograph reveals elevated diaphragms, and pulmonary function tests shows reduced lung volumes • Diaphragmatic myopathy restricted lung volume • a/w Anti Ro Ab TONY SCARIA 2010 KMC Cardiac Manifestations in SLE • 1. Pericarditis is the most frequent cardiac manifestation; • 2. Endocarditis of Libman-sacks • 3. AR, MR • 4. Risk for myocardial infarction • 5. Myocarditis • 6. Endocarditis Overall most important cause of death n SLE  cardiac failure TONY SCARIA 2010 KMC
11-03-2018 Cutaneous manifestation in SLE • 1. Systemic rash • 2. Discoid rash • 3. Subacute cutaneous lupus erythematosus (LE) Rash is a photosensitive. • 4. Urticaria • 5. Lichen planus-like dermatitis • 6. Bullae. • 7. Small painful ulcerations on the oral or nasal mucosa are common in SLE. • 8. Tin-tack sign • 1. Also known as carpet tack sign , is a useful clinical feature in diagnosing discoid lupus erythematosus. • 2. Hyperkeratotic scale extending into the follicular infundibulum creates keratotic spikes when viewed from the scale’sundersurface, resembling a carpet tack. TONY SCARIA 2010 KMC Tin tack sign TONY SCARIA 2010 KMC
11-03-2018 Hematologic • 1. Coombs positive hemolytic anemia • 2. Lymphopenia • 3. Thrombo cytopenia • 4. Anti phospholipid syndrome – • lupus anti coagulant and anti cardiolipin are present. It consists of thrombocytopenia, recurrent fetal loss, recurrent venous or arterial clotting. TONY SCARIA 2010 KMC Not a feature of SLE • Lung cavitation • Joint deformity • Raynauds phenomenon TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Significance of anti Ro Ab • anti-Ro, which indicates increased risk for • neonatal lupus, • sicca syndrome, and • Sub acute cutaneous lupus erythematosus (SCLE) TONY SCARIA 2010 KMC
11-03-2018 • Women with child-bearing potential and SLE should be screened for • aPL to r/o APLA • anti-Ro to r/o risk for neonatal lupus TONY SCARIA 2010 KMC • Complement level are reduced in SLE. Immune complex diseases associated with hypocomplementemia • 1. SLE • 2. Vasculitis • 1. Hypocomplementemic urticarial vasculitis • 2. Polyarteritis nodosa (especially hepatitis Bassociated (necrotizing vasculitis) • 3. Glomerulonephritis • 1. Post-streptococcal • 2. Membranoproliferative • 3. Cryoglobulinemia (types II and III) • 4. Bacterial endocarditis • 5. Serum sickness TONY SCARIA 2010 KMC
11-03-2018 Drug induced lupus erythematosus • Procainamide and hydralazine are most common offenders. • Most patients do not have symptoms of lupus erythematosus. • high frequency of antihistone antibodies. • multiple organs may be affected renal and CNS involvement, usually does not occur. • Sex ratio is equal (in contrast to idiopathic SLE where females are affected more commonly) TONY SCARIA 2010 KMC Drug induced lupus • Acebutolol • Methyldopa • Asparaginase • Phenolphthalein • Barbiturates • Phenytoin • Bleomycin • Procainamide • Hydralazine TONY SCARIA 2010 KMC
11-03-2018 Drugs associated with SLE are • 1. Antiarrhythmics → (Procainamide, disopyramide, propafenone) • 2. Antihypertensives → (Hydralazine,. ACE inhibitors, and Beta blockers) • 3. Antithyroid → (Propylthiouracil) • 4. An psycho cs → (Chlorpromazine and lithium) • 5. An convulsant → (Carbamazepine and phenytoin) • 6. An bio cs → (Minocycline and Macrolides, INH) • 7. An hyperlipidemic → (Lovasta n, Simvasta n) TONY SCARIA 2010 KMC drug induced SLE • 1. Anti histone antibodies are positive • 2. Anti dsDNA is usually absent • 3. Renal involvement is not a feature or very uncommon • 4. Polyserositis is a feature • 5. Rash is very common • 6. Features disappear on discontinuation of drugs. TONY SCARIA 2010 KMC
11-03-2018 • Medication • 1. NSAIDs, salicylates (St. Joseph's aspirin (low dose aspirin) • 2. Topical glucocorticoids • 3. Topical sunscreens • 4. Hydroxychloroquine (quinacrine can be added or substituted) • 5. DHEA (dehydroepiandrosterone) • 6. Methotrexate (for dermatitis, arthritis) • 7. Glucocorticoids, oral • 8. Methylprednisolone sodium succinate, IV • 9. Cyclophosphamide IV • 10. Mycophenolate mofetil or mycophenolic acid • 11. Azathioprine • 12. Belimumab • 13. Rituximab TONY SCARIA 2010 KMC Rx • Low dose aspirin (St Joseph aspirin) therapy is used in • 1. Pre-eclampsia • 2. Coronary artery disease. • 3. Anti phospholipid syndrome • 4. IUGR • Indication steroid therapy in SLE • 1. Nephritis • 2. Thrombocytopenic purpura • 3. Hemolytic anemia • 4. Pericarditis • 5. Convulsion Low dose aspirin not used in SLE without APLS TONY SCARIA 2010 KMC
11-03-2018 New diagnostic modalities in SLE • Cell bound compliment activation product (CB – CAP) • Platelet bound compliment product (P-C4d) • Diagnostic marker in Urine. • 1. Endothelin 1 • 2. Lipocalcin 2 • 3. U-MCP-1 • 4. Hepcidin TONY SCARIA 2010 KMC Systemic sclerosis TONY SCARIA 2010 KMC
11-03-2018 Systemic sclerosis (scleroderma) • F>>M, 30–50 Yrs TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Mirovascular injury  hallmark feature of SScl TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 • Anti topoisomerase I (Anti Scl 70) • Widespread skin involvement • Rapid progression and early visceral involvement Diffuse scleroderma • Anticentromere Ab • Limited skin involvement, confined to fingers, forearms • When skin alone is involved then it is known as Morphea. Limited or localized scleroderma TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 • CREST syndrome which is a combination of • 1. Calcinosis: subcutaneous calcifications of fingers and along extensor surface of forearm • 2. Raynaud phenomenon • 3. Esophageal dysmotility • 4. Sclerodactyly (skin thickening limited to fingers) • 5. Telangiectasia (dilated capillaries) TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 • 1. Antinuclear antibodies. • 2. Antibodies to single stranded RNA • 3. Anti - Scl - 70 (antitopoisomerase) seen in generalize scleroderma. • a/w pulmonary fibrosis • Peripheral vascular disease • 4. Anticentromere antibody. These autoantibodies are seen in patient with CREST syndrome. • 5. Antiendothelial cell antibody. TONY SCARIA 2010 KMC • 1. RNA polymerase III – Associated with acute onset scleroderma renal crises & cancer • 2. U3-RNP • 3. U1-RNP TONY SCARIA 2010 KMC
11-03-2018 Morphea • isolated patches of hardened skin • no internal organ involvement • Diagnosis is by clinical suspicion, presence of autoantibodies (especifcally anti-centromere and anti-scl70/anti-topoisomerase antibodies) and occasionally by biopsy TONY SCARIA 2010 KMC C/F TONY SCARIA 2010 KMC
11-03-2018 Raynauds phenomenon present in 80 % of patients TONY SCARIA 2010 KMC • Pulmonary Manifestations • 1. Pulmonary interstitial fibrosisa/w diffuse scleroderma • 2. Pulmonary hypertension a/w CREST SYNDROME • 3. RVF TONY SCARIA 2010 KMC
11-03-2018 GIT manifestations • involvement of the lower two thirds of the esophagus resulting in loss of esophageal peristalsis and dysphagia • Gastric antral vascular ectasia (GAVE) in the antrum • "watermelon stomach" due to their endoscopic appearance • recurrent episodes of gastrointestinal bleeding, resulting in chronic unexplained anemia. TONY SCARIA 2010 KMC • Musculoskeletal Manifestations • arthralgia and a mild non – erosive inflammatory arthritis. • Inflammation of synovium with hypertrophy and hyperplasia of synovial soft tissues. • Closely reminiscent of rheumatoid arthritis but joint destruction is not common. TONY SCARIA 2010 KMC
11-03-2018 • Renal Involvement sudden renal crisis a/w RNA polymerase III Ab • 1. There is intimal hyperplasia of the interlobular arteries, fibrinoid necrosis of the afferent arterioles, including the glomerular tuft, and thickening of the glomerular basement membrane. • 2. This change results in the development of renin induced hypertension. • 3. Sudden renal failure can occur TONY SCARIA 2010 KMC Rx • *Management* • 1. D – Penicillamine • 2. Glucocorticoids • 3. Hypertension, • alpha – methyldopa, calcium channel blockers or ACEI, • as these drugs may alleviate Raynaud’s phenomenon. • Beta – blockers are contraindicated in treating hypertension, as may precipitate Raynaud’s phenomenon TONY SCARIA 2010 KMC
11-03-2018 sjogrens syndrome TONY SCARIA 2010 KMC Sjogren’s syndrome • chronic, slowly progressive autoimmune disease • lymphocytic infiltration of the exocrine glands resulting in xerostomia and dry eyes TONY SCARIA 2010 KMC
11-03-2018 Triad of sjogrens syndrome 1. Dry eye 2. Dry mouth 3. B/L parotid enlargement TONY SCARIA 2010 KMC Type of sjogrens syndrome • Idiopathic • Less common • associated with: HLA - DR - 3 Primary • Occurs in a/w AutoImmune d/s • More common  60 % • Usually associated with: HLA - DR - 4 Secondary TONY SCARIA 2010 KMC
11-03-2018 Primary sjogrens syndrome Glandular involvement •Involves exocrine glands •Xerostomia •Keratoconjunctivitis sicca Extraglandular •Arthralgia / arthritis (MC extraglandular involvement ) •Marginal zone B cell lymphomaMikulics syndrome  enlargement of lacrimal & salivary gland from any cause TONY SCARIA 2010 KMC Features of 1* psjogrens • 1. Arthralgia/arthritis • 2. Lung involvement • 3. Liver involvement • 4. Peripheral neuropathy • 5. Raynaud's phenomenon • 6. Vasculitis • 7. Lymphoma • 8. Myositis • 9. Lymphadenopathy • 10. Kidney involvement • 11. Splenomegaly TONY SCARIA 2010 KMC
11-03-2018 Secondary sjogren • *Causes of secondary Sjögren’s syndrome* • 1. Rheumatoid arthritis (most common cause) • 2. SLE • 3. Scleroderma • 4. Mixed connective tissue disease • 5. Primary biliary cirrhosis • 6. Vasculitis • 7. Chronic active hepatitis • 8. Polymyositis • 9. Hashimoto's thyroiditis • 10. Interstitial pulmonary fibrosis TONY SCARIA 2010 KMC • RHEUMATOID FACTOR • Ig M auto Ab that binds self IgG • presence of autoantibodies to • Ro/SS – A and • La/SS – B antigens • Antibodies to α - fodrin (120kDa), • a salivary gland- specific protein may be present TONY SCARIA 2010 KMC
11-03-2018 • Diagnostic Criteria for Sjögren’s Syndrome • 1. Dry eyes • 2. Dry mouth • 3. Positive Schirmer’s I test • 4. Histopathology - Focus score 1 in a minor salivary gland biopsy • 5. Reduce salivary flow • 6. Antibodies to Ro (SS – A) or La(SS – B), antinuclear antibodies, or rheumatoid factor • Probable Sjögren’s syndrome : three items are present. • Definite Sjögren’s syndrome: four or more items are present. TONY SCARIA 2010 KMC Diagnosis • Biopsy of the lip (to examine minor salivary glands) TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Focus score in sjorens syndrome • Focus score • (the number of mononuclear cell infiltrates containing at least 50 inflammatory cells in a 4 mm2 glandular section) of greater than 1 is probably the single most important test result in the diagnosis of the oral component in SS TONY SCARIA 2010 KMC
11-03-2018 Rx • 1. Pilocarpine given orally sicca manifestations. • 2. Hydroxychloroquine arthralgias. • 3. Glucocorticoids or other immunosuppressive agents (i.e, cyclophosphamide) • extraglandular manifestations. TONY SCARIA 2010 KMC Inflammatory myopathies TONY SCARIA 2010 KMC
11-03-2018 Inflammatory myopathies • Immunologically mediated • Most common a/w systemic sclerosis TONY SCARIA 2010 KMC Types of inflammatory myopathies • dermatomyositis, • polymyositis, • inclusion body myositis TONY SCARIA 2010 KMC
11-03-2018 Dermatomyositis • Diagnostic Criteria: • (4 out of 5, for dermatomyositis cutaneous changes must present) • 1. Proximal symmetric progressive muscle weakness • 2. Elevated muscle enzyme • CPK, AST, LDH • 3. Consistent electromyography (EMG) • to locate the affected muscles • 4. Consistent muscle biopsy • Perifasicular atrophy of muscle fibres+ inflammation • 5. Cutaneous disease TONY SCARIA 2010 KMC Perifasicualr atrophy with inflammation TONY SCARIA 2010 KMC
11-03-2018 Cutaneous features of dermatomyositis Major cutaneous criteria •Gottron’s papule •Gottron’s sign •Heliotrope rash Minor criteria •V sign •Shawl sign • holster sign TONY SCARIA 2010 KMC Heliotrope rash • Violaceous erythema with edema over eyelids, periorbital region TONY SCARIA 2010 KMC
11-03-2018 Gottrons papule • lilac or violaceous papules on knuckle, dorsa of hands. TONY SCARIA 2010 KMC Gottrons sign • Violaceous erythema with edema over shoulder, arms, forearms. TONY SCARIA 2010 KMC
11-03-2018 Mechanics hand • Symmetric hyperkeratosis along ulnar aspect of thumb and radial aspect of fingers TONY SCARIA 2010 KMC Shawl sign TONY SCARIA 2010 KMC
11-03-2018 V sign in dermatomyositis TONY SCARIA 2010 KMC • Poikiloderma: - Atrophy of skin, hypopigmentatio n, dilated blood vessels over trunk TONY SCARIA 2010 KMC
11-03-2018 • Holster sign  hip TONY SCARIA 2010 KMC Nail-fold telangiectasia • In dermatomyositis TONY SCARIA 2010 KMC
11-03-2018 Muscle involvement in dermatomyositis • Proxmial muscle weakness • symmetrical and bilateral • Progressive weakness TONY SCARIA 2010 KMC • Calcinosis cutis occurs in juvenile form of dermatomyositis TONY SCARIA 2010 KMC
11-03-2018 Auto Ab in dermatomyositis Anti-Jo1 (histidyl tRNA synthetase) antibody • polymyositis with interstitial lung disease, pulmonary fibrosis • Nonerosive arthritis • Skin rash Anti Mi2 Ab • Gottron papule • Heliotrope rash Anti P55/P140 Ab • Paraneoplastic dermatomyositis • Juvenile dermatomyositis TONY SCARIA 2010 KMC Treatment of skin diseases: • 1. Sun-protection: Topical steroids • 2. Antimalarials: Hydroxychloroquine (below 6.5 mg/kg/day), same as for cutaneous LE. • 3. Systemic steroids are sometimes used but generally more effective in controlling myositis than cutaneous manifestations Systemic steroids may cause steroid myopathy with prolonged use, leading to paradoxical decrease in muscle weakness following steroid dosage reduction. TONY SCARIA 2010 KMC
11-03-2018 Inclusion body myositis TONY SCARIA 2010 KMC Inclusion body myositis rimmed vacuoles (basophilic rimming) TONY SCARIA 2010 KMC
11-03-2018 Inclusion body myositis • MC inflammatory myopathy in patients older than 65 yrs • Presents with slowly progressive distal muscle weakness • cN1A antibodies are present in this myositis TONY SCARIA 2010 KMC Mixed Connective Tissue Disorder (MCTD) • overlap syndrome characterized by a combination of clinical features similar to those of SLE, scleroderma & polymyositis • high titers of circulating anti U1 RNP antibodies • Treatment • 1. Salicylates and other NSAID • 2. Glucocorticoids TONY SCARIA 2010 KMC
11-03-2018 Causes of b/l parotid enlargement Viral • 1. Mumps • 2. Influenza • 3. Epstein Barr virus • 4. Coxsackie virus A • 5. CMV • 6. HIV Metabolic • 1. Diabetes mellitus • 2. Hyperlipoproteinemia • 3. Chronic pancreatitis • 1. Amyloidosis • 2. Sjögren’s syndrome • 3. Sarcoidosis Endocrine • Gonadal hypofunction TONY SCARIA 2010 KMC HLA A3 Hemochromatosis HLA DR2 • Narcolepsy • Goodpasture’s syndrome HLA-DR3 • Autoimmune hepatitis, • Primary Sjögren syndrome-1, • Diabetes mellitus type-1, • Systemic lupus erythematosus • Dermatitis herpetiformis HLA- DR4 • Rheumatoid arthritis, • Diabetes mellitus type 1 • secondary sjoogrens HLAB5 Behcets HLAB27 • Ankylosing spondylitis, • Psoriaticarthritis, • Reiter's syndrome, • Reactive arthritis(Yersinia,salmonella,gonococci), • JRA (pauci articular) HLA-B47 • 21-hydroxylase deficiency TONY SCARIA 2010 KMC
11-03-2018 • Multiple sclerosis- HLA DR2 • Narcolepsy- HLA DR2 • Goodpasture’s syndrome- HLA DR2 • Sjogrens- HLA DR3 • Dermatitis herpetiformis- HLA DR3 • Celiac disease- HLA DR3 • Rheumatoid arthritis- HLA DR4 • Behcets disease – HLA B5 • Congenital adrenal hyperplasia - HLA B47 • SLE- HLA DR2,DR3 • Type 1 Diabetes – HLA DR2,DR3,DR4 • Hyperthyroidism – HLA B8,DR3 • Myasthenia gravis- HLA B8,DR3 • Tuberculoid leprosy- HLA B8,DR3 • VKH syndrome- HLA DW 15,DR4 TONY SCARIA 2010 KMC Synovium TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Arthritis TONY SCARIA 2010 KMC Cells of synovium 2 types TONY SCARIA 2010 KMC
11-03-2018 Type A & B cells of synovium TONY SCARIA 2010 KMC Synovial fluid • Non Newtonian • Thixotropic fluid • Hyalouronic acid  viscosity & lubricating property TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Articular cartilage • Mainly articular cartilage • contain type II collagen • Avascular  nutrition derived from synovium • No nerve supply • No perichondrium • Lacks ability to repair & regenerate TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Superficial zone • Densely packed collagen • Flattened ellipsoid shaped chondrocytes with their major axis parallel to joint surface Transition zone Middle zone • Radial zone • Chondrocytes are synthetically most active Calcified cartilage • Low metabolic activity TONY SCARIA 2010 KMC Arthritis TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC • Non inflammatory arthritis • OA • Traumatic arthritis • Pigmented villnodular synovitis TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Infective arthritis • Tom smith arthritis • Septic arthritis TONY SCARIA 2010 KMC distinguish b/w transient synovitis & septic arthritis • Transient arthritis is non specific inflammation of the synovium, related to infection or trauma. • Septic arthritis is bacterial infection of the synovium of the joint. • Modified Kochers criteria • Fever > 38.5 • ESR > 40 mm/hr • WBC > 12,000 cells/mL • CRP > 20 mg/dL • Inability to bear weight on the affected limb • More the features  more chance of septic arthritis TONY SCARIA 2010 KMC
11-03-2018 Tom smith arthritis • Septic arthritis of hip in an infant • Chondrolysis • Presents with limp & unstable gait • Shortening of leg  supratrochanteric shortening • Telescopy • Increased movement in all directions • X ray • Complete absence of head & NOF TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Septic arthritis • Pyogenic arthritis • Mc cause of bony ankylosis • Mc organism over all  staph aureus by hematogenous route • Mc in neonates group B streptococci • Mc organism in sexually active young adults  N gonorrhoea • Chondrolysis is commn in infancy • Best method of diagnosis  aspiration of foci • Rx • Sx  arthroscopy & wash to focus along with IV Abx TONY SCARIA 2010 KMC Causes of migratory arthritis • Lymes disease • Rheumatic fever • Disseminated gonococcal infection TONY SCARIA 2010 KMC
11-03-2018 Causes of polyarticular symmetric arthritis • Rheumatoid arthritis • Systemic lupus erythematosus • Viral arthritis • Hepatitis, • parvovirus B19 • In females working in nursery • EBV TONY SCARIA 2010 KMC Causes of monoarticular arthritis • Osteoarthritis • Gout • Pseudogout (Maybe polyarticular) • Septic arthritis TONY SCARIA 2010 KMC
11-03-2018 Causes of oligoarticular asymmetric arthritis • Spondyloarthropathies • Osteoarthritis TONY SCARIA 2010 KMC Osteoarthritis • Disorder of synovial joint • Non inflammatory • Progressive softening & disintegration of articular cartilage TONY SCARIA 2010 KMC
11-03-2018 OA / advanced degenerative joint ds • Degenerative • Asymmetric • Localised to one part of joint • c/c non inflammatory progressive softening & disintegration of articular cartilage • Destruction of articular cartilage  main event • Osteophytosis • Growth of new cartilage & bone @ joint margins • Capsular fibrosis TONY SCARIA 2010 KMC • Most common joint disease • Vastus medialis (quadriceps) • Mc involved muscle in OSA • Wasting of quadriceps • Etiology • Excessive loading inspite of normal articular cartilage & subchondral bone • Normal load inspite of weak cartilage & bone TONY SCARIA 2010 KMC
11-03-2018 • Mc knee joint is involved • MC deformity is genu varus • Sparing of ankle elbow & wrist TONY SCARIA 2010 KMC • Age is the most important powerful risk factor TONY SCARIA 2010 KMC
11-03-2018 • Primary pathological process of OA involves articular cartilage TONY SCARIA 2010 KMC Classification Primary • Idiopathic • Mc involves knee joint • Localised • Mono/pauci articular • Generalised • Poly articular > 3 joint Secondary • Secondary to • Trauma • # • Developmental/congenital disorders • Gout / AVN/ endocrine disorders TONY SCARIA 2010 KMC
11-03-2018 • Polyarticular / generalised OA • Mc form • Most commonly involves DIP • Classical monoarticular /pauciarticular OA • One or two large weight bearing bone • Knee>>> hip • Symptomatic OA of knee is more common in men than in women TONY SCARIA 2010 KMC • Hip  rare • More common in older men • Hand • More commonly involved in elderly female • DIP PIP • Non nodal erosive IP arthritis • Carpo metacarpal joint of thumb TONY SCARIA 2010 KMC
11-03-2018 • OA characteristically involves • DIP  Heberdens node *** • PIP  Bouchards node • H is more distal than B in alphabets • Base of thumb 1st MCP • Other MCP & wrist are spared TONY SCARIA 2010 KMC • Foot • Hallux valgus/rigidus • Hammer toes TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Radiological features of OA • 'asymmetric narrowing of joint space‘ • Due to wearing of cartilage • EARLIEST SIGN • sclerosis of the subchondral bone under the area of cartilage loss, • d/t new bone formation • cysts close to the joint surface and • osteophytes at the margins of the joint • Loose bodies & deformities TONY SCARIA 2010 KMC
11-03-2018 • In knee joint  medial joint space is affected first  genu varus TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Osteo arthritis - Womack grading. • Stage i • there is softening of the cartilage if the surfaces of the knee. This may not show on X-ray. • Stage ii • the cartilage starts to wear away and shows as a narrowed joint space. • Stage iii • osteophytes are seen. Stage 2 and 3 may merge and be difficult to separate. • Stage iv • "bone-on-bone" with very narrow joint space, lots of osteophytes, and distortion of the joint TONY SCARIA 2010 KMC Osteo arthritis - Outerbridge  cartilage Classification • Grade 0: normal cartilage; • Grade I: cartilage with softening and swelling; • Grade II: a partial-thickness defect with fissures on the surface that do not reach subchondral bone or exceed 1.5 cm in diameter; • Grade III: fissuring to the level of subchondral bone in an area with a diameter more than 1.5 cm; • Grade IV, exposed subchondral bone TONY SCARIA 2010 KMC
11-03-2018 Rx • i. Initial management of • 1. Physical therapy • 2. Bracing, orthoses, ambulatory aids • 3. NSAID, glucosamine, chondroitin, intraarticular injections of steroid or hyaluronic acid, and analgesics. TONY SCARIA 2010 KMC • Glucosamine • Increases lubrication • Chondroitin sulphate • Increases lubrication • Diacerin • Reduces IL -1 induced cartilage damage • S – adenosyl methionine(SAM) • Reduces pain & cartilage damage • Tetracycline • Inhibits metalloproteinase • Not routinely used TONY SCARIA 2010 KMC
11-03-2018 • Sx • In severe disabling pain • Interfering with day to day activities • Grade 2- 5 radiological evidence TONY SCARIA 2010 KMC • ii. Arthroscopic débridement • active, older adults with mild-to-moderate OA of the knee after conservative treatment • Only in presence of mechanical symptom like locking / loose bodies in X ray • iii. High tibial osteotomy • It is a well-established procedure for the treatment of unicompartmental OA of the knee. • To decrease weight distribution in that compartment TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC • Type I to III conservative management • Non pharmacological • Life style modification /physiotherapy/bracing • pharmacological • NSAIDS/intrarticular injection of steroid • Type IV & V  surgery • Arthroscopic wash out / debridement • Realignment Sx • High tibial osteotomy • Unicompartmental arthroplasty • TKR TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Indications for arthroplasty Total knee replacememt • Pain in severe OA • Deformities • Severe pain from chondrocalcinosis & pseudogout & severe patellofemoral OA • Tricompartmental OA Total hip replacement • Alleviation of pain • Improve function of hip • Arthritis • AVN • Bone tumours involving acetabulum & proximal femur TONY SCARIA 2010 KMC Rheumatoid arthritis • c/c immunologically mediated • Persistent inflammatrory synovitis • Symmetrically involving peripheral joints • Cartilage damage /bone erosion /change in joint integrityTONY SCARIA 2010 KMC
11-03-2018 • Pathogenesis • Women (30-50 yrs) • HLA DR4 DW4 • Initial site  synovial membrane TONY SCARIA 2010 KMC Stages • Initiation phase • d/t non specific inflmn • Amplification phase • d/t T cell activation • c/c inflammatory phase • Due to cytokines IL- 1 , TNF alpha & IL - 6 TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC • Score of > 6 = definite RA TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Pathogenesis of RA TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Manifestations of RA • Erosive arthritis • Joint swelling tenderness & limitation of motion • Pain aggravated by movement  Mc manifestation • Axial involvement • Limited to Atlantoaxial joint (cervical spine) • Bakers cyst • Extension of inflamed synovium in to popliteal space TONY SCARIA 2010 KMC
11-03-2018 Involvement of hand • Z deformity • Radial deviation of wrist • Ulnar deviation of fingers of hand *** • pathognomonic TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 At DIP mallet deformity Rupture of extensor tendon at DIP TONY SCARIA 2010 KMC Swan neck deformity • Extension of PIP & compensatory flexion of DIP TONY SCARIA 2010 KMC
11-03-2018 boutonniere deformity • Flexion of PIP & extension of DIP TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Intrinsic plus hand deformity in RA (d/t contracture of lumbricals) Assessed by bunnels test TONY SCARIA 2010 KMC • Eversion of hind foot deformity • @ subtalar joint • plantar subluxation of metatarsal head • Hammer toes • Widening of forefoot • Claw toe TONY SCARIA 2010 KMC
11-03-2018 Windswept deformity • Valgus deformity of toes in foot & varus in other TONY SCARIA 2010 KMC Hammer toe / contracted toe • Deformity of 2nd 3rd 4th toe • Causing it to be permanently bent • Mallet toe • Similar condition affecting DIP TONY SCARIA 2010 KMC
11-03-2018 Claw toe • Dorsiflexion of proximal phalanx on lesser MTP joint • Combined with flexion of DIP & PIP TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Rheumatoid arthritis of spine • Involves usually atlantoaxial joint • Dens erosion through foramen magnum • May encroach spine TONY SCARIA 2010 KMC Knee joint in RA • Valgus • Triple deformity • Flexion + • Posterior subluxn + • External rotation TONY SCARIA 2010 KMC
11-03-2018 Rheumatoid nodules • in pressure areas such as the elbow, finger joints, Achilles tendon and occipital part of scalp TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 rheumatoid vasculitis • Polyneuropathy • Mononeuritis multiplex • Digital gangrene • MI TONY SCARIA 2010 KMC Skin involvement in RA • Vasculitis  skin necrosis • Malignant rheumatoid arthritis TONY SCARIA 2010 KMC
11-03-2018 Eye – • a. Episcleritis • b. Scleritis • c. Conjunctivitis • d. Scleromalacia (Rupture of the Globe) can occurs Uveitis is not a feature TONY SCARIA 2010 KMC Neurological symptoms – • 1. Peripheral neuropathy – due to vasculitis. • 2. Cord compression – due to atlanto axial subluxation • 3. Nerve entrapment – due to proliferative, synovitis • (Carpal tunnel syndrome due to median nerve compression, tarsaltunnel syndrome due to Posterior tibial nerve compression). TONY SCARIA 2010 KMC
11-03-2018 Pleuropulmonary manifestation of RA • Pleuritis • Pleural effusion • Mainly on left side • Low pleural fluid sugar • Arteritis • Pneumonitis • Pleuropulmonary nodule TONY SCARIA 2010 KMC Caplans syndrome • Pneumoconiosis • RA • Lung nodules TONY SCARIA 2010 KMC
11-03-2018 Multiple Lung nodules are also found in Caplan’s syndrome Wegeners granulomatosis Silicosis TONY SCARIA 2010 KMC Pericarditis • Constrictive pericarditis • Asymptomatic TONY SCARIA 2010 KMC
11-03-2018 • RENAL INVOLVEMENT IS NOT A FEATURE OF RA • EVEN IF OCCURS D/T AMYLOIDOSIS OR DRUG INDUCED TONY SCARIA 2010 KMC Radiologic changes in RA • Soft tissue swelling • Joint effusion • Symmetrical involvement • Juxta aticular osteopenia • With in weeks • Loss of articular cartilage & erosion • Narrowing of joint space • Subluxation & ankyloses • Lack of hypertrophic changes • Like sclerosis / osteophyte TONY SCARIA 2010 KMC
11-03-2018 Rheumatoid factor • Is used in diagnosis & prognosis • Not used for screening • About 80% of individuals with rheumatoid arthritis have autoantibodies to the Fc portion of autologous IgG (rheumatoid factors). • These are mostly IgM antibodies • RF & Ig combine to form d/s process • correlates with ds activity • decline in the level of rheumatoid factor is associated with reduced clinical disease activity TONY SCARIA 2010 KMC • RF is also positive in • 5% healthy people • SLE • c/c liver ds • Sjogrens syndrome • Pulm fibrosis • IMN • Hep B • TB • Leprosy • Syphilis • SABE • Leishmaniasis • Malaria • schistosomiasis TONY SCARIA 2010 KMC
11-03-2018 Diagnosis • Normocytic normochromic anemia • Raised ESR CRP & ceruloplasmin • Anti CCP Is positive in RA • Most specific Ab • Synovial fluid analysis • Turbid • Decreased viscosity • Increased protein content • Decreased sugar • WBC 5000- 50000/ml TONY SCARIA 2010 KMC Marker of active ds • Anemia of c/c ds • Increased ESR CRP & platelet count • Decreased synovial fluid viscosity TONY SCARIA 2010 KMC
11-03-2018 Feltys syndrome TONY SCARIA 2010 KMC Feltys syndrome • Complement level is decreased • • Cause of leukopenia in RA is • 1. Drug induce – most common • 2. Felty’s syndrome • 3. T-LGL TONY SCARIA 2010 KMC
11-03-2018 Causes of leukopenia • Feltys syndrome • Drug induced  most common • T- IGL TONY SCARIA 2010 KMC Haematology in RA • Anemia in RA Normocytic normochromia • d/t ineffective erythropoiesis • GI bleed a/w NSAIDs • eosinophilia in RA • Severe systemic ds • Large B cell lymphoma  lymphoma in RA TONY SCARIA 2010 KMC
11-03-2018 Eye • Scleritis • Episcleritis • Conjunctivitis • Scleromalacia (rupture of globe) • Uveitis is not a feature TONY SCARIA 2010 KMC Neurological system • CNS is not directly involved • Peripheral neuropathy • d/t vasculitis • Cord ompression • d/t atlanto-axial subluxation • Nerve entrapment • d/t proliferative + synovitis  carpel tunnel syndrome • Tarsal tunnel syndrome • Posterior tibial nerve compression TONY SCARIA 2010 KMC
11-03-2018 Poor prognosis • High titres of RF • C-RP • Haptoglobulin • Subcutaneous nodules • Radiographic evidence • Life expectancy is decreased by 3-7 yrs TONY SCARIA 2010 KMC Complications of RA • Amyloidosis  NS • Septic arthritis • By staph aureus TONY SCARIA 2010 KMC
11-03-2018 • NSAIDs • Etodolac  chiral NSAID for Rx of RA • Licofelone • Inhibits both lipoxygenase & cyclooxygenase of AA metabolism • No GI s/e • Analgesic anti inflammatory /antihistaminic • Flupirtine • Selective K+ channel opener • NMDA receptor antagonist properties • Non NSAID non opid nonsteroidal analgesic TONY SCARIA 2010 KMC DMARDs • Slow down progression of ds • Methotrexate *** • Mc used • D – pencillamine • Sulfasalazine • Gold • HCQ TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC s/e of DMARDs • Gold/pencillamine • NS • Pancytopenia • HCQ • Retinopathy • Sulphasalazine • Skin rash • Methotrexate • Liver dysfunction TONY SCARIA 2010 KMC
11-03-2018 Glucocorticoid rx in RA • Vasculitis • Mononeuritis multiplex • Pericarditis • Perforating eye lesion • Pleural effusion TONY SCARIA 2010 KMC Anti TNF alpha drugs • Adalimumab • Certrolizumab • Etanarcept • Golimumab • infliximab TONY SCARIA 2010 KMC
11-03-2018 s/e anti TNF alpha drugs • Serious infections • Disseminated TB • Pancytopenia • Demyelination disorders • Hypersensitivity • Exacerbation of CHF • Hypersensitivity • Severe liver disease • SLE • NO INCREASED RISK OF MALIGANANCY • Avoided in patients with hypersensitivity / active infection TONY SCARIA 2010 KMC Tocilizumab • Monoclonal Ab directed against IL – 6 receptor cell membrane • IL – 6  involved in pathogenesis of RA  joint inflammation & damage TONY SCARIA 2010 KMC
11-03-2018 IL-1 receptor antagonist • ANAKINRA TONY SCARIA 2010 KMC Rituximab • Anti CD – 20 Ab • Monoclonal Ab • Attaches to B cell TONY SCARIA 2010 KMC
11-03-2018 ibritumomab tiuxetan • Murine monoclonal Ab labelled with yttrium 90 or indium 111 • In cases refractory to rituximab TONY SCARIA 2010 KMC Abatacept  fusion protein TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Trastuzumab • Anti – HER2 /neu Ab • Monoclonal Ab directed against HER2 /neu receptor tyrosine kinase against breast cancer with over expression of this factor TONY SCARIA 2010 KMC
11-03-2018 Immunosuppressants • Cyclophosphamide • Leflunomide • Azathioprine • Cyclosporine TONY SCARIA 2010 KMC Orthopedic procedures in patients with RA • Tenosynovectomy • Tendon repair • Synovectomy • arthroplasty • Arthrodesis TONY SCARIA 2010 KMC
11-03-2018 • 1. Anemia – *Normocytic normochromic anemia* and is due to ineffective • erythropoiesis • 2. ESR – It is increased in all patients with active RA. • 3. C – reactive protein and ceruloplasmin are increased • 4. Synovial fluid analysis – • 1. Turbid • 2. Decreased viscosity , • 3. Increase protein content • 4. Decrease glucose concentration • 5. WBC count is 5000 – 50000 per ml TONY SCARIA 2010 KMC Psoriatric arthritis TONY SCARIA 2010 KMC
11-03-2018 Psoriatic arthritis • Seronegative polysynovitis erosive • HLA DR7 • HLA CW6 • Mc presentation  assymmetric oligoarthritis of large joints • High incidence of sacroilitis & spondylitis • HLA B27 • RF negative TONY SCARIA 2010 KMC Psoriatic arthritis • In 30% psoriatic patients • Skin lesions (early) joint involvement (late) • Nail changes+ • PsA features include • DIP joint involvement • Dactylitis (sausage digitals) • Arthritis mutilans(severe destructive arthritis) • Corneal nodules • Liver damage • Rx • MTX ,etanarcept TONY SCARIA 2010 KMC
11-03-2018 CF • 5 patterns of joint involvement • Arthritis of DIP joint • Asymmetrical oligoarthritis  MC • Symmetrical poly arthritis similar to RA • Axial involvement  spine & sacroiliac joint • Arthritis mutilans TONY SCARIA 2010 KMC • Shortening of digits (d/t underlying osteolysis)TELESCOPING • Pencil in cup deformity + TONY SCARIA 2010 KMC
11-03-2018 • 1. DIP involvement, & classical pencil in cup deformity • 2. Marginal erosion with adjacent bony proliferation, (whiskering) • 3. Small joint ankylosis • 4. Osteolysis of phalangeal and metacarpal bone, with telescoping of digits • 5. Periostitis and proliferative new bone at sites of enthesitis • 6. Severe cervical spine involvement with atlantoaxial subluxation, but relative sparing of thoracolumbar spine, and paravertebral ossification. TONY SCARIA 2010 KMC Pencil in cup deformity TONY SCARIA 2010 KMC
11-03-2018 Pencil in cup deformity TONY SCARIA 2010 KMC Whiskering + TONY SCARIA 2010 KMC
11-03-2018 Saucage digitals d/t dactylitis TONY SCARIA 2010 KMC arthritis mutilans TONY SCARIA 2010 KMC
11-03-2018 • Telescoping • Underlying osteolysis  telescoping • Nail changes • Less tender than RA • Conjunctivitis & Ch uveitis • Fibrous & bony ankylosis • PsA>> RA TONY SCARIA 2010 KMC Radiological signs • Opera glass hand (telescoping) • Telescoping of bones in to its neighbours with shortening of digits • Pencil in cup sign • Tapering of proximal phalanx and bony prolifern of distal phalanx TONY SCARIA 2010 KMC
11-03-2018 Radiological signs in PsA • Whiskering • Marginal erosions with adjacent bony proliferation • Mouse ear TONY SCARIA 2010 KMC Opera glass hand sign TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Criteria for PsA caspar TONY SCARIA 2010 KMC
11-03-2018 Rx of PsA • Methotrexate is drug of choice • Anti TNF - a agents • etanercept and infliximab are newer drugs and are effective even in longstanding resistent PsA cases (to previous therapy) and extensive skin lesion • Other effective agents are sulfasalazine, cyclosporine retinoic acid & psoralen & UV-A (PUVA) TONY SCARIA 2010 KMC Seronegative spondyloarthropathies • Onset <40 years • HLA B 27 positive • Presence of uveitis • Absence of auto Ab • Inflammatory of arthritis of spine / large joints TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC AS TONY SCARIA 2010 KMC
11-03-2018 Ankylosing spondylitis / marie – strumpell/bechtew ds • Genetically determined • Generalised c/c inflammatory ds • Primarily affects axial skeleton • With variable involvement of shoulder or hip TONY SCARIA 2010 KMC • Primary pathology • Enthesitis • Site of ligamentous attachment to bone • Primarily I pelvis & spine • Prominent edema of adjacent bonemarrow fb ossification • Peripheral arthritis of AS • TNF alpha & cytokines TONY SCARIA 2010 KMC
11-03-2018 • AS • HLA B27 +ve • M:F=3:1 • Earliest manifestation  sacroilitis (enthesitis+ synovitis) • Sacroiliac joint mainly on iliac side • Extra articular manifestation • a/c anterior uveitis *** mc • cardiac manifestation  AR , 3rd degree heart block , conductive defects • GI involvement  inflammation of colon / ileum (IBD) • Restrictive lung ds TONY SCARIA 2010 KMC • M:F=2:1 • 15 – 25 yrs • dull pain, insidious in onset, felt deep in lower lumbar or gluteal region, accompanied by low back morning stiffness of up to few hour duration that improves with activity and returns following period of inactivity TONY SCARIA 2010 KMC
11-03-2018 Musculoskeletal system • Enthesopathy • Inflammation of tendon & ligament attached to it • Synovitis of diarthroidal joints • Sacroiliac joint • Facet foint of vertebrae • Costovertebral joint • Inflammation of fibroosseous junction of syndesmotic joints • SI joint ligaments • Manubrium sternii • Pubic symphysis • Intervertebral disc TONY SCARIA 2010 KMC 1. ? Mark posture • Hyperkyphosis of thoracic spine & loss of lumbar lordosis 2. Sacroilitis TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC • Should increase by atleast 5 cm in on fexion • <5cm  restriction of flexion TONY SCARIA 2010 KMC
11-03-2018 Shobers test TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Limitation of chest expansion • d/t costovertebral joint involvement • <2cm  AS TONY SCARIA 2010 KMC
11-03-2018 Eye involvement in AS • Iritis + TONY SCARIA 2010 KMC Spine • Loss of lumbar lordosis (Straightening) • Enthesitis – Increased Blood flow – Cause absorption by • Erosions of anterior corners of vertebral body causing squaring of vertebral body. • Delicate syndesmophyte with “vertical orientation bridging vertebral bodies” “(horizontal in degenerative spine disease)”. • Bamboo spine, syndesmophytes and paravertebral ossifcation TONY SCARIA 2010 KMC
11-03-2018 • Xray • Loss of lumbar lordosis • Sclerosis of anterior corners of vertenbral body • Squarring of vertebrae • Bamboo spine TONY SCARIA 2010 KMC Sacro iliac joint • Blurring of margins • Juxta-articular sclerosis • Erosions cause pseudowidening • Obliteration of joint (fbrous followed by bony ankylosis) TONY SCARIA 2010 KMC
11-03-2018 • Complication • Mc  spine # even with minor trauma TONY SCARIA 2010 KMC • Rx • Phenylbutazone  most effective drug • Causes aplastic anemia • Hence reserved for resistant cases • indomethacin • Sulfasalazine + FA antagonist • Anti TNF alpha therapy • Infliximab / etanarcept TONY SCARIA 2010 KMC
11-03-2018 Diffuse idiopathic skeletal hypertrostosis (DISH) Ankylosing spondylitis Syndesmophytes Nonmarginal (paravertebral) Marginal X ray Flowing candle wax • Bamboo spine • Squaring of vertebral bodies Radiodensity Increased Radiolucent HLA B8 B27 a/w DM + - Age Elderly Young TONY SCARIA 2010 KMC Flowing wax like appearance in DISH TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Neuropathic joint d/s / charcot’s joint • Progressive destructive arthritis • d/t loss of sensation & proprioception  repeated trauma  bone & cartilage damage • Hence Less pain is experienced than anticipated • DM ***>>tabes dorsalis • Radiological features • Same as OA • Diagnosis • MRI & bone scan • Using indium 111 labelled WBC / IgG (to differentiate from OM increased uptake in osteomyelitis but not in neuropathic joint) TONY SCARIA 2010 KMC
11-03-2018 d/s associated with charcots joint • DM*** • Midtarsal >>tarsometatarsal>>metatarsophalangeal>>ankle >> knee & spine • Tabes dorsalis • Associated with lightening pin in lower limbs • Knee joint is affected • Meningomyelocele • Ankle / intertarsal • Peroneal muscular atrophy • Syringomyelia • Shoulder elbow wrist cervical spine • Leprosy • Amyloidosis • c/c alcoholism • Metatrsophalangeal / IP TONY SCARIA 2010 KMC Radiological finding in charcots joint • Joint space narrowing • Joint becomes progressively enlarged from bony overgrowth and synovial effusion • swelling, instability, laxity & progressive deformity TONY SCARIA 2010 KMC
11-03-2018 Tumbling building block spine • In charcot spine TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Rx of charcots joint • limitation of joint movements by bracing or casting, joint debridement (arthrocentesis) and fusion of joint (arthrodesis) TONY SCARIA 2010 KMC Hemophilic arthropathy • Order of joint involvement • Knee >> ankle >> elbow>> shoulder>>hip • Position of joint • Flexion + abduction + external rotation • Avoid joint aspiration & arthroscopy TONY SCARIA 2010 KMC
11-03-2018 • Hemophilia • X-linked recessive disorder • manifested only in males but females are carriers • characterized by the absence or • defciency of factor VIII (hemophilia A or classical hemophilia)  85% • factor IX (hemophilia B or Christmas disease) TONY SCARIA 2010 KMC Haemarthrosis • Ankle is most commonly involved in children • Joint assumes position of minimal discomfort and minimal intra-articular pressure, which is, • flexion (30–65°) abduction (15°) and lateral rotation (15°) in hip; and slight flexion in knee. • Pain, warmth, boggy swelling, tenderness and limited movements cause it to resemble with low grade infection. TONY SCARIA 2010 KMC
11-03-2018 • Clotting Factors • • <1% ~ Spontaneous Hemorrhage • • 1-5% - Hemorrhage on Mild Trauma • • >5% Hemorrhage on Signifcant Trauma TONY SCARIA 2010 KMC Haemophilic pseudotumour • In severe haemophiliacs • Progressive cystic swelling caused by uncontrolled haemorrhage with in confined space • Haematoma increases in size  pressure necrosis • Mc location  thigh >> abdomen > pelvis • Iliopsoas >>> quadriceps TONY SCARIA 2010 KMC
11-03-2018 Intramuscular bleeding • Bleeding into muscle is less common but equally harmful can cause muscle necrosis, reactive fbrosis and joint contractures. • Mc site of bleeding in LL • Quadriceps • Mc site of bleeding in UL • Deltoid • Bleeding into iliopsoas & retroperitoneum • Resembles APPENDICITIS TONY SCARIA 2010 KMC Radiologic features of haemophilic arthritis • Soft tissue swelling • Juxtaarticular osteopenia (not sclerosis) • Overgrowth & osteoporosis of epiphyses • Marginal erosions • Subchondral cyst • Widened intercondylar notch of femur • Trochlear notch of ulna • Enlarged proximal radius • Squarring distal end of patella • Total loss of joint space • Fibrous ankylosis TONY SCARIA 2010 KMC
11-03-2018 Rx • Factor replacement in haemophilia • Joint aspiration (in severe cases) • Ice & analgesics  to relieve pain • Rest • Compressive bandage TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC Gout • 1st MTP  mc involved joint • Monosodium crystals  • negatively birefringent • Needle shaped • Tophi • Extensor aspect of elbow • Pinna • 1st MTP TONY SCARIA 2010 KMC
11-03-2018 Polyarticular gout TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Acute gout • First episode @ night • With dramatic joint pain & swelling • Skin is shiny red  similar to cellulitis TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TOPHI in Xray TONY SCARIA 2010 KMC MARTELS SIGN or G (GOUT) SIGN • Punched out with over hanging bone • Chronic tophaceous gout • -Cystic changes and well-defined erosions described as punched-out lytic lesions with overhanging bony edges (Martels sign, or G sign for gout), associated with soft tissue calcified masses TONY SCARIA 2010 KMC
11-03-2018 MSU crystals in polarised light TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Pseudogout • Calcium pyrophosphate crystals • Rhomboid /rectangular • Positively refringent • Moderate pain • Large joints  knee joint – mc involved joint • Chondrocalcinosis on xray TONY SCARIA 2010 KMC Pseudogout is a/w • Hypothyroidism • Hemochromatosis • Hyperaprathyroidism (primary) • Hypomagensemia • Hypophosphatemia • Wilsons ds TONY SCARIA 2010 KMC
11-03-2018 • Chondrocalcinosis • Radiological hall mark of pseudogout • Linear /multiple punactate in menisci TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
11-03-2018 Milwaukee shoulder • More common in female • With h/o trauma • d/t deposition of calcium hydroxyapatite crystal • Mc in shoulder • Definitive diagnosis of apatite arthropathy depends on identification of crystals from synovial fluid or tissue • X ray  multiple loose bodies • Rx • Intraarticular steroid TONY SCARIA 2010 KMC Reiter syndrome • Reactive arthritis • Seronegative spondyloarthropathies • HLA B27 + • Classic triad • Conjunctivitis / uveitis • Arthritis large joints in LL • Urethritis • Circinate balanitis + • Keratoderma blenorrhagica TONY SCARIA 2010 KMC
11-03-2018 • Bacteria causing Reactive Arthritis • 1. Salmonella • 2. Shigella MC ORGANISM • 3. Campylobacter • 4. Yersinia • 5. Chlamydia. MC cause following urethritis • Reiter disease can occur in epidemic form TONY SCARIA 2010 KMC Rx of reiters ds • 1. Analgesics • 2. Local corticosteroids are useful in the case of iritis • 3. The nonspecific urethritis is treated with a short course of • Tetracycline. TONY SCARIA 2010 KMC
11-03-2018 Ankylosis Bony ankylosis • Pyogenic arthritis of all joints *** mc cause • Tuberculous arthritis of spine Fibrous ankylosis • Tuberculous arthrotis of hip / knee /small joints TONY SCARIA 2010 KMC Transient synovitis of hip • aka Observation hip • Self limiting condition usually lasts only a short time • aka Coxalgia fugax /coxitis serosa • Mc of hip pain in children < 10 yrs (3-12 yrs of age) • With rapid onset of pain • Limited to groin or may be referred to thigh • limping • Antalgic gait • limited range of movement • Gaurded rotation of hip • Tenderness at extremes of motion • Following viral URTI • Usually u/l TONY SCARIA 2010 KMC
11-03-2018 FABER  flexion abduction external rotation TONY SCARIA 2010 KMC X ray • Widened joint space medially • Bone density is normal TONY SCARIA 2010 KMC
11-03-2018 USG • Mild joint effusion • USG guided aspiration • For definitive cytological diagnosis • To r/o septic arthritis TONY SCARIA 2010 KMC Rx • Observation • Traction TONY SCARIA 2010 KMC
11-03-2018 Chondrocalcinosis • Knee joint  MC involved in menisci • Causes • All causes Pseudogout  most important cause • Alkaptonuria • Wilsons ds TONY SCARIA 2010 KMC Arthrtis Erosive arthritis • RA • Gout • Hyperparathyroidiam Nonerosive • SLE • OA TONY SCARIA 2010 KMC
11-03-2018 Spondyloarthrosis • Aka Seronegative arthritis • 1. Ankylosing spondylitis • 2. Reiter’s disease • 3. Reactive Arthritis • 4. Psoriatic arthritis • 5. Juvenile rheumatoid arthritis (JRA) • 6. Inflammatory bowel disease TONY SCARIA 2010 KMC Features of spondyloarthrosis • 1. Seronegativity (rheumatoid factor –ve) • 2. HLA B27 associated • 3. Axial arthritis: Pathology in spine and sacroiliitis. • 4. Asymmetrical large-joint oligoarthritis (i.e <5 joints) or monoarthritis. • 5. Enthesitis: Inflammation of the site of insertion of tendon or ligament into bone, eg. Plantar fasciitis, Achillis tendonitis, costochondritis. • 6. Extra-articular manifestations eg anterior uveitis, aortic regurgitation TONY SCARIA 2010 KMC
11-03-2018 Arthritis in children TONY SCARIA 2010 KMC Acute(<2 weeks ) •Rheumatic fever •Septic arthritis Sub acute(2-6 weeks) •Reactive arthritis Chronic(>6 weeks) •JRATONY SCARIA 2010 KMC
11-03-2018 JIA Juvenile rheumatoid arthritis Juvenile psoriatic arthritis Juvenile enthesitis related arthritis TONY SCARIA 2010 KMC Juvenile rheumatoid arthritis • Diagnostic criteria TONY SCARIA 2010 KMC
11-03-2018 JRA pauciarticular Type I Type II Polyarticular Polyarticular RA positive Polyartciular RA negative Systemic onset • ANA +ve • RF –ve • Eye involvement with iridocyclitis glaucoma cataract • HLA DR5 & DRW8 • ANA –ve • RF-ve • Iritis only (not iridocyclitis) • HLA B27 • ANA –ve • RF-ve • Iritis only (not iridocyclitis) • HLA B27 • Symmetrical joint involvement with uveitis & rheumatoid nodule • RA factor + • ANA +ve • No rheumatoid nodule • No RF • Fever with ras • lymphadenopathy HSM • RF –ve • Uveitis (iridocyclitis ) absent TONY SCARIA 2010 KMC RF is positive only in polyarticular JRA TONY SCARIA 2010 KMC
11-03-2018 • No iritis or uveitis in systemic type & polyarticular RA negative TONY SCARIA 2010 KMC Rx • NSAIDs • Naproxen • Ibuprofen • Indomethacin TONY SCARIA 2010 KMC

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Rheumatology revison notes

  • 1.
  • 2.
    11-03-2018 TONY SCARIA 2010 KMC Homogeneous •Ab against ds DNA nucleosomes & histone Peripheral rim • Ab against ds DNA & to nuclear envelope protein TONY SCARIA 2010 KMC
  • 3.
    11-03-2018 Speckled pattern • Abagainst nuclear non DNA contents • Sm Ag  SLE • SSA & SSB sjogren syndrome • Ribonucleopattern  Mixed connective tissue ds Nucleolar pattern • Ab to RNA in systemic sclerosis Centromere pattern • Anticentromere Ab in systemic sclerosis TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 4.
    11-03-2018 SLE TONY SCARIA 2010KMC • Differential Diagnosis of Rash over face (Malar) • 1. Non erythematous- • 1. Chloasma • 2. Erythematous- • 1. SLE • 2. Photosensitivity reaction • 3. Contact dermatitis Sparing of nasolabial fold TONY SCARIA 2010 KMC
  • 5.
    11-03-2018 TONY SCARIA 2010KMC TONY SCARIA 2010 KMC
  • 6.
    11-03-2018 TONY SCARIA 2010KMC TONY SCARIA 2010 KMC
  • 7.
  • 8.
    11-03-2018 Wire loop lesion •Formed by subendothelial deposits • Seen in class 3,4 and 5 • Most commonly a/w class 4 • It indicates active ds & poor prognosis TONY SCARIA 2010 KMC Adverse prognosis • Factors associated with Adverse Prognosis and High Risk of Renal Progression in Lupus Nephritis • Hypertension • Hypocomplementemia • Thrombocytopenia • High Anti Ds DNA antibody titres • Pregnancy • Nephritic urinary sediment • Azotemia • Proteinuria • Decreased GFR TONY SCARIA 2010 KMC
  • 9.
    11-03-2018 Better prognosis • Presenceof anti LA (SSB) indicate lower involvement of kidney in SLE TONY SCARIA 2010 KMC • Most common cause of death in SLE with in 10 years is renal failure (followed by infection) • But most common over all cause is cardiac failure TONY SCARIA 2010 KMC
  • 10.
    11-03-2018 Muskuloskeletal system • Mcsystem to be involved in SLE • Non erosive arthritis TONY SCARIA 2010 KMC CNS • Psychosis a/w anti P Ab (antiribosomal P Ab) TONY SCARIA 2010 KMC
  • 11.
    11-03-2018 Lungs involvement inSLE • Pleuritis and pleural effusions (the most common pulmonary manifestations). • Alveolar injury (edema and hemorrhage) • Chronic interstitial fibrosis. • Shrinking lung syndrome • The chest radiograph reveals elevated diaphragms, and pulmonary function tests shows reduced lung volumes • Diaphragmatic myopathy restricted lung volume • a/w Anti Ro Ab TONY SCARIA 2010 KMC Cardiac Manifestations in SLE • 1. Pericarditis is the most frequent cardiac manifestation; • 2. Endocarditis of Libman-sacks • 3. AR, MR • 4. Risk for myocardial infarction • 5. Myocarditis • 6. Endocarditis Overall most important cause of death n SLE  cardiac failure TONY SCARIA 2010 KMC
  • 12.
    11-03-2018 Cutaneous manifestation inSLE • 1. Systemic rash • 2. Discoid rash • 3. Subacute cutaneous lupus erythematosus (LE) Rash is a photosensitive. • 4. Urticaria • 5. Lichen planus-like dermatitis • 6. Bullae. • 7. Small painful ulcerations on the oral or nasal mucosa are common in SLE. • 8. Tin-tack sign • 1. Also known as carpet tack sign , is a useful clinical feature in diagnosing discoid lupus erythematosus. • 2. Hyperkeratotic scale extending into the follicular infundibulum creates keratotic spikes when viewed from the scale’sundersurface, resembling a carpet tack. TONY SCARIA 2010 KMC Tin tack sign TONY SCARIA 2010 KMC
  • 13.
    11-03-2018 Hematologic • 1. Coombspositive hemolytic anemia • 2. Lymphopenia • 3. Thrombo cytopenia • 4. Anti phospholipid syndrome – • lupus anti coagulant and anti cardiolipin are present. It consists of thrombocytopenia, recurrent fetal loss, recurrent venous or arterial clotting. TONY SCARIA 2010 KMC Not a feature of SLE • Lung cavitation • Joint deformity • Raynauds phenomenon TONY SCARIA 2010 KMC
  • 14.
    11-03-2018 TONY SCARIA 2010 KMC Significanceof anti Ro Ab • anti-Ro, which indicates increased risk for • neonatal lupus, • sicca syndrome, and • Sub acute cutaneous lupus erythematosus (SCLE) TONY SCARIA 2010 KMC
  • 15.
    11-03-2018 • Women withchild-bearing potential and SLE should be screened for • aPL to r/o APLA • anti-Ro to r/o risk for neonatal lupus TONY SCARIA 2010 KMC • Complement level are reduced in SLE. Immune complex diseases associated with hypocomplementemia • 1. SLE • 2. Vasculitis • 1. Hypocomplementemic urticarial vasculitis • 2. Polyarteritis nodosa (especially hepatitis Bassociated (necrotizing vasculitis) • 3. Glomerulonephritis • 1. Post-streptococcal • 2. Membranoproliferative • 3. Cryoglobulinemia (types II and III) • 4. Bacterial endocarditis • 5. Serum sickness TONY SCARIA 2010 KMC
  • 16.
    11-03-2018 Drug induced lupuserythematosus • Procainamide and hydralazine are most common offenders. • Most patients do not have symptoms of lupus erythematosus. • high frequency of antihistone antibodies. • multiple organs may be affected renal and CNS involvement, usually does not occur. • Sex ratio is equal (in contrast to idiopathic SLE where females are affected more commonly) TONY SCARIA 2010 KMC Drug induced lupus • Acebutolol • Methyldopa • Asparaginase • Phenolphthalein • Barbiturates • Phenytoin • Bleomycin • Procainamide • Hydralazine TONY SCARIA 2010 KMC
  • 17.
    11-03-2018 Drugs associated withSLE are • 1. Antiarrhythmics → (Procainamide, disopyramide, propafenone) • 2. Antihypertensives → (Hydralazine,. ACE inhibitors, and Beta blockers) • 3. Antithyroid → (Propylthiouracil) • 4. An psycho cs → (Chlorpromazine and lithium) • 5. An convulsant → (Carbamazepine and phenytoin) • 6. An bio cs → (Minocycline and Macrolides, INH) • 7. An hyperlipidemic → (Lovasta n, Simvasta n) TONY SCARIA 2010 KMC drug induced SLE • 1. Anti histone antibodies are positive • 2. Anti dsDNA is usually absent • 3. Renal involvement is not a feature or very uncommon • 4. Polyserositis is a feature • 5. Rash is very common • 6. Features disappear on discontinuation of drugs. TONY SCARIA 2010 KMC
  • 18.
    11-03-2018 • Medication • 1.NSAIDs, salicylates (St. Joseph's aspirin (low dose aspirin) • 2. Topical glucocorticoids • 3. Topical sunscreens • 4. Hydroxychloroquine (quinacrine can be added or substituted) • 5. DHEA (dehydroepiandrosterone) • 6. Methotrexate (for dermatitis, arthritis) • 7. Glucocorticoids, oral • 8. Methylprednisolone sodium succinate, IV • 9. Cyclophosphamide IV • 10. Mycophenolate mofetil or mycophenolic acid • 11. Azathioprine • 12. Belimumab • 13. Rituximab TONY SCARIA 2010 KMC Rx • Low dose aspirin (St Joseph aspirin) therapy is used in • 1. Pre-eclampsia • 2. Coronary artery disease. • 3. Anti phospholipid syndrome • 4. IUGR • Indication steroid therapy in SLE • 1. Nephritis • 2. Thrombocytopenic purpura • 3. Hemolytic anemia • 4. Pericarditis • 5. Convulsion Low dose aspirin not used in SLE without APLS TONY SCARIA 2010 KMC
  • 19.
    11-03-2018 New diagnostic modalitiesin SLE • Cell bound compliment activation product (CB – CAP) • Platelet bound compliment product (P-C4d) • Diagnostic marker in Urine. • 1. Endothelin 1 • 2. Lipocalcin 2 • 3. U-MCP-1 • 4. Hepcidin TONY SCARIA 2010 KMC Systemic sclerosis TONY SCARIA 2010 KMC
  • 20.
    11-03-2018 Systemic sclerosis (scleroderma) •F>>M, 30–50 Yrs TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 21.
    11-03-2018 Mirovascular injury hallmark feature of SScl TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 22.
    11-03-2018 • Anti topoisomeraseI (Anti Scl 70) • Widespread skin involvement • Rapid progression and early visceral involvement Diffuse scleroderma • Anticentromere Ab • Limited skin involvement, confined to fingers, forearms • When skin alone is involved then it is known as Morphea. Limited or localized scleroderma TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 23.
    11-03-2018 • CREST syndromewhich is a combination of • 1. Calcinosis: subcutaneous calcifications of fingers and along extensor surface of forearm • 2. Raynaud phenomenon • 3. Esophageal dysmotility • 4. Sclerodactyly (skin thickening limited to fingers) • 5. Telangiectasia (dilated capillaries) TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 24.
    11-03-2018 • 1. Antinuclearantibodies. • 2. Antibodies to single stranded RNA • 3. Anti - Scl - 70 (antitopoisomerase) seen in generalize scleroderma. • a/w pulmonary fibrosis • Peripheral vascular disease • 4. Anticentromere antibody. These autoantibodies are seen in patient with CREST syndrome. • 5. Antiendothelial cell antibody. TONY SCARIA 2010 KMC • 1. RNA polymerase III – Associated with acute onset scleroderma renal crises & cancer • 2. U3-RNP • 3. U1-RNP TONY SCARIA 2010 KMC
  • 25.
    11-03-2018 Morphea • isolated patchesof hardened skin • no internal organ involvement • Diagnosis is by clinical suspicion, presence of autoantibodies (especifcally anti-centromere and anti-scl70/anti-topoisomerase antibodies) and occasionally by biopsy TONY SCARIA 2010 KMC C/F TONY SCARIA 2010 KMC
  • 26.
    11-03-2018 Raynauds phenomenon presentin 80 % of patients TONY SCARIA 2010 KMC • Pulmonary Manifestations • 1. Pulmonary interstitial fibrosisa/w diffuse scleroderma • 2. Pulmonary hypertension a/w CREST SYNDROME • 3. RVF TONY SCARIA 2010 KMC
  • 27.
    11-03-2018 GIT manifestations • involvement ofthe lower two thirds of the esophagus resulting in loss of esophageal peristalsis and dysphagia • Gastric antral vascular ectasia (GAVE) in the antrum • "watermelon stomach" due to their endoscopic appearance • recurrent episodes of gastrointestinal bleeding, resulting in chronic unexplained anemia. TONY SCARIA 2010 KMC • Musculoskeletal Manifestations • arthralgia and a mild non – erosive inflammatory arthritis. • Inflammation of synovium with hypertrophy and hyperplasia of synovial soft tissues. • Closely reminiscent of rheumatoid arthritis but joint destruction is not common. TONY SCARIA 2010 KMC
  • 28.
    11-03-2018 • Renal Involvementsudden renal crisis a/w RNA polymerase III Ab • 1. There is intimal hyperplasia of the interlobular arteries, fibrinoid necrosis of the afferent arterioles, including the glomerular tuft, and thickening of the glomerular basement membrane. • 2. This change results in the development of renin induced hypertension. • 3. Sudden renal failure can occur TONY SCARIA 2010 KMC Rx • *Management* • 1. D – Penicillamine • 2. Glucocorticoids • 3. Hypertension, • alpha – methyldopa, calcium channel blockers or ACEI, • as these drugs may alleviate Raynaud’s phenomenon. • Beta – blockers are contraindicated in treating hypertension, as may precipitate Raynaud’s phenomenon TONY SCARIA 2010 KMC
  • 29.
    11-03-2018 sjogrens syndrome TONY SCARIA2010 KMC Sjogren’s syndrome • chronic, slowly progressive autoimmune disease • lymphocytic infiltration of the exocrine glands resulting in xerostomia and dry eyes TONY SCARIA 2010 KMC
  • 30.
    11-03-2018 Triad of sjogrenssyndrome 1. Dry eye 2. Dry mouth 3. B/L parotid enlargement TONY SCARIA 2010 KMC Type of sjogrens syndrome • Idiopathic • Less common • associated with: HLA - DR - 3 Primary • Occurs in a/w AutoImmune d/s • More common  60 % • Usually associated with: HLA - DR - 4 Secondary TONY SCARIA 2010 KMC
  • 31.
    11-03-2018 Primary sjogrens syndrome Glandularinvolvement •Involves exocrine glands •Xerostomia •Keratoconjunctivitis sicca Extraglandular •Arthralgia / arthritis (MC extraglandular involvement ) •Marginal zone B cell lymphomaMikulics syndrome  enlargement of lacrimal & salivary gland from any cause TONY SCARIA 2010 KMC Features of 1* psjogrens • 1. Arthralgia/arthritis • 2. Lung involvement • 3. Liver involvement • 4. Peripheral neuropathy • 5. Raynaud's phenomenon • 6. Vasculitis • 7. Lymphoma • 8. Myositis • 9. Lymphadenopathy • 10. Kidney involvement • 11. Splenomegaly TONY SCARIA 2010 KMC
  • 32.
    11-03-2018 Secondary sjogren • *Causesof secondary Sjögren’s syndrome* • 1. Rheumatoid arthritis (most common cause) • 2. SLE • 3. Scleroderma • 4. Mixed connective tissue disease • 5. Primary biliary cirrhosis • 6. Vasculitis • 7. Chronic active hepatitis • 8. Polymyositis • 9. Hashimoto's thyroiditis • 10. Interstitial pulmonary fibrosis TONY SCARIA 2010 KMC • RHEUMATOID FACTOR • Ig M auto Ab that binds self IgG • presence of autoantibodies to • Ro/SS – A and • La/SS – B antigens • Antibodies to α - fodrin (120kDa), • a salivary gland- specific protein may be present TONY SCARIA 2010 KMC
  • 33.
    11-03-2018 • Diagnostic Criteriafor Sjögren’s Syndrome • 1. Dry eyes • 2. Dry mouth • 3. Positive Schirmer’s I test • 4. Histopathology - Focus score 1 in a minor salivary gland biopsy • 5. Reduce salivary flow • 6. Antibodies to Ro (SS – A) or La(SS – B), antinuclear antibodies, or rheumatoid factor • Probable Sjögren’s syndrome : three items are present. • Definite Sjögren’s syndrome: four or more items are present. TONY SCARIA 2010 KMC Diagnosis • Biopsy of the lip (to examine minor salivary glands) TONY SCARIA 2010 KMC
  • 34.
    11-03-2018 TONY SCARIA 2010 KMC Focusscore in sjorens syndrome • Focus score • (the number of mononuclear cell infiltrates containing at least 50 inflammatory cells in a 4 mm2 glandular section) of greater than 1 is probably the single most important test result in the diagnosis of the oral component in SS TONY SCARIA 2010 KMC
  • 35.
    11-03-2018 Rx • 1. Pilocarpinegiven orally sicca manifestations. • 2. Hydroxychloroquine arthralgias. • 3. Glucocorticoids or other immunosuppressive agents (i.e, cyclophosphamide) • extraglandular manifestations. TONY SCARIA 2010 KMC Inflammatory myopathies TONY SCARIA 2010 KMC
  • 36.
    11-03-2018 Inflammatory myopathies • Immunologicallymediated • Most common a/w systemic sclerosis TONY SCARIA 2010 KMC Types of inflammatory myopathies • dermatomyositis, • polymyositis, • inclusion body myositis TONY SCARIA 2010 KMC
  • 37.
    11-03-2018 Dermatomyositis • Diagnostic Criteria: •(4 out of 5, for dermatomyositis cutaneous changes must present) • 1. Proximal symmetric progressive muscle weakness • 2. Elevated muscle enzyme • CPK, AST, LDH • 3. Consistent electromyography (EMG) • to locate the affected muscles • 4. Consistent muscle biopsy • Perifasicular atrophy of muscle fibres+ inflammation • 5. Cutaneous disease TONY SCARIA 2010 KMC Perifasicualr atrophy with inflammation TONY SCARIA 2010 KMC
  • 38.
    11-03-2018 Cutaneous features ofdermatomyositis Major cutaneous criteria •Gottron’s papule •Gottron’s sign •Heliotrope rash Minor criteria •V sign •Shawl sign • holster sign TONY SCARIA 2010 KMC Heliotrope rash • Violaceous erythema with edema over eyelids, periorbital region TONY SCARIA 2010 KMC
  • 39.
    11-03-2018 Gottrons papule • lilacor violaceous papules on knuckle, dorsa of hands. TONY SCARIA 2010 KMC Gottrons sign • Violaceous erythema with edema over shoulder, arms, forearms. TONY SCARIA 2010 KMC
  • 40.
    11-03-2018 Mechanics hand • Symmetrichyperkeratosis along ulnar aspect of thumb and radial aspect of fingers TONY SCARIA 2010 KMC Shawl sign TONY SCARIA 2010 KMC
  • 41.
    11-03-2018 V sign indermatomyositis TONY SCARIA 2010 KMC • Poikiloderma: - Atrophy of skin, hypopigmentatio n, dilated blood vessels over trunk TONY SCARIA 2010 KMC
  • 42.
    11-03-2018 • Holster sign hip TONY SCARIA 2010 KMC Nail-fold telangiectasia • In dermatomyositis TONY SCARIA 2010 KMC
  • 43.
    11-03-2018 Muscle involvement indermatomyositis • Proxmial muscle weakness • symmetrical and bilateral • Progressive weakness TONY SCARIA 2010 KMC • Calcinosis cutis occurs in juvenile form of dermatomyositis TONY SCARIA 2010 KMC
  • 44.
    11-03-2018 Auto Ab indermatomyositis Anti-Jo1 (histidyl tRNA synthetase) antibody • polymyositis with interstitial lung disease, pulmonary fibrosis • Nonerosive arthritis • Skin rash Anti Mi2 Ab • Gottron papule • Heliotrope rash Anti P55/P140 Ab • Paraneoplastic dermatomyositis • Juvenile dermatomyositis TONY SCARIA 2010 KMC Treatment of skin diseases: • 1. Sun-protection: Topical steroids • 2. Antimalarials: Hydroxychloroquine (below 6.5 mg/kg/day), same as for cutaneous LE. • 3. Systemic steroids are sometimes used but generally more effective in controlling myositis than cutaneous manifestations Systemic steroids may cause steroid myopathy with prolonged use, leading to paradoxical decrease in muscle weakness following steroid dosage reduction. TONY SCARIA 2010 KMC
  • 45.
    11-03-2018 Inclusion body myositis TONYSCARIA 2010 KMC Inclusion body myositis rimmed vacuoles (basophilic rimming) TONY SCARIA 2010 KMC
  • 46.
    11-03-2018 Inclusion body myositis •MC inflammatory myopathy in patients older than 65 yrs • Presents with slowly progressive distal muscle weakness • cN1A antibodies are present in this myositis TONY SCARIA 2010 KMC Mixed Connective Tissue Disorder (MCTD) • overlap syndrome characterized by a combination of clinical features similar to those of SLE, scleroderma & polymyositis • high titers of circulating anti U1 RNP antibodies • Treatment • 1. Salicylates and other NSAID • 2. Glucocorticoids TONY SCARIA 2010 KMC
  • 47.
    11-03-2018 Causes of b/lparotid enlargement Viral • 1. Mumps • 2. Influenza • 3. Epstein Barr virus • 4. Coxsackie virus A • 5. CMV • 6. HIV Metabolic • 1. Diabetes mellitus • 2. Hyperlipoproteinemia • 3. Chronic pancreatitis • 1. Amyloidosis • 2. Sjögren’s syndrome • 3. Sarcoidosis Endocrine • Gonadal hypofunction TONY SCARIA 2010 KMC HLA A3 Hemochromatosis HLA DR2 • Narcolepsy • Goodpasture’s syndrome HLA-DR3 • Autoimmune hepatitis, • Primary Sjögren syndrome-1, • Diabetes mellitus type-1, • Systemic lupus erythematosus • Dermatitis herpetiformis HLA- DR4 • Rheumatoid arthritis, • Diabetes mellitus type 1 • secondary sjoogrens HLAB5 Behcets HLAB27 • Ankylosing spondylitis, • Psoriaticarthritis, • Reiter's syndrome, • Reactive arthritis(Yersinia,salmonella,gonococci), • JRA (pauci articular) HLA-B47 • 21-hydroxylase deficiency TONY SCARIA 2010 KMC
  • 48.
    11-03-2018 • Multiple sclerosis-HLA DR2 • Narcolepsy- HLA DR2 • Goodpasture’s syndrome- HLA DR2 • Sjogrens- HLA DR3 • Dermatitis herpetiformis- HLA DR3 • Celiac disease- HLA DR3 • Rheumatoid arthritis- HLA DR4 • Behcets disease – HLA B5 • Congenital adrenal hyperplasia - HLA B47 • SLE- HLA DR2,DR3 • Type 1 Diabetes – HLA DR2,DR3,DR4 • Hyperthyroidism – HLA B8,DR3 • Myasthenia gravis- HLA B8,DR3 • Tuberculoid leprosy- HLA B8,DR3 • VKH syndrome- HLA DW 15,DR4 TONY SCARIA 2010 KMC Synovium TONY SCARIA 2010 KMC
  • 49.
  • 50.
    11-03-2018 Arthritis TONY SCARIA 2010KMC Cells of synovium 2 types TONY SCARIA 2010 KMC
  • 51.
    11-03-2018 Type A &B cells of synovium TONY SCARIA 2010 KMC Synovial fluid • Non Newtonian • Thixotropic fluid • Hyalouronic acid  viscosity & lubricating property TONY SCARIA 2010 KMC
  • 52.
  • 53.
    11-03-2018 Articular cartilage • Mainlyarticular cartilage • contain type II collagen • Avascular  nutrition derived from synovium • No nerve supply • No perichondrium • Lacks ability to repair & regenerate TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 54.
    11-03-2018 Superficial zone • Densely packed collagen •Flattened ellipsoid shaped chondrocytes with their major axis parallel to joint surface Transition zone Middle zone • Radial zone • Chondrocytes are synthetically most active Calcified cartilage • Low metabolic activity TONY SCARIA 2010 KMC Arthritis TONY SCARIA 2010 KMC
  • 55.
  • 56.
    11-03-2018 TONY SCARIA 2010 KMC •Non inflammatory arthritis • OA • Traumatic arthritis • Pigmented villnodular synovitis TONY SCARIA 2010 KMC
  • 57.
  • 58.
    11-03-2018 Infective arthritis • Tomsmith arthritis • Septic arthritis TONY SCARIA 2010 KMC distinguish b/w transient synovitis & septic arthritis • Transient arthritis is non specific inflammation of the synovium, related to infection or trauma. • Septic arthritis is bacterial infection of the synovium of the joint. • Modified Kochers criteria • Fever > 38.5 • ESR > 40 mm/hr • WBC > 12,000 cells/mL • CRP > 20 mg/dL • Inability to bear weight on the affected limb • More the features  more chance of septic arthritis TONY SCARIA 2010 KMC
  • 59.
    11-03-2018 Tom smith arthritis •Septic arthritis of hip in an infant • Chondrolysis • Presents with limp & unstable gait • Shortening of leg  supratrochanteric shortening • Telescopy • Increased movement in all directions • X ray • Complete absence of head & NOF TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 60.
    11-03-2018 Septic arthritis • Pyogenicarthritis • Mc cause of bony ankylosis • Mc organism over all  staph aureus by hematogenous route • Mc in neonates group B streptococci • Mc organism in sexually active young adults  N gonorrhoea • Chondrolysis is commn in infancy • Best method of diagnosis  aspiration of foci • Rx • Sx  arthroscopy & wash to focus along with IV Abx TONY SCARIA 2010 KMC Causes of migratory arthritis • Lymes disease • Rheumatic fever • Disseminated gonococcal infection TONY SCARIA 2010 KMC
  • 61.
    11-03-2018 Causes of polyarticularsymmetric arthritis • Rheumatoid arthritis • Systemic lupus erythematosus • Viral arthritis • Hepatitis, • parvovirus B19 • In females working in nursery • EBV TONY SCARIA 2010 KMC Causes of monoarticular arthritis • Osteoarthritis • Gout • Pseudogout (Maybe polyarticular) • Septic arthritis TONY SCARIA 2010 KMC
  • 62.
    11-03-2018 Causes of oligoarticularasymmetric arthritis • Spondyloarthropathies • Osteoarthritis TONY SCARIA 2010 KMC Osteoarthritis • Disorder of synovial joint • Non inflammatory • Progressive softening & disintegration of articular cartilage TONY SCARIA 2010 KMC
  • 63.
    11-03-2018 OA / advanceddegenerative joint ds • Degenerative • Asymmetric • Localised to one part of joint • c/c non inflammatory progressive softening & disintegration of articular cartilage • Destruction of articular cartilage  main event • Osteophytosis • Growth of new cartilage & bone @ joint margins • Capsular fibrosis TONY SCARIA 2010 KMC • Most common joint disease • Vastus medialis (quadriceps) • Mc involved muscle in OSA • Wasting of quadriceps • Etiology • Excessive loading inspite of normal articular cartilage & subchondral bone • Normal load inspite of weak cartilage & bone TONY SCARIA 2010 KMC
  • 64.
    11-03-2018 • Mc kneejoint is involved • MC deformity is genu varus • Sparing of ankle elbow & wrist TONY SCARIA 2010 KMC • Age is the most important powerful risk factor TONY SCARIA 2010 KMC
  • 65.
    11-03-2018 • Primary pathologicalprocess of OA involves articular cartilage TONY SCARIA 2010 KMC Classification Primary • Idiopathic • Mc involves knee joint • Localised • Mono/pauci articular • Generalised • Poly articular > 3 joint Secondary • Secondary to • Trauma • # • Developmental/congenital disorders • Gout / AVN/ endocrine disorders TONY SCARIA 2010 KMC
  • 66.
    11-03-2018 • Polyarticular /generalised OA • Mc form • Most commonly involves DIP • Classical monoarticular /pauciarticular OA • One or two large weight bearing bone • Knee>>> hip • Symptomatic OA of knee is more common in men than in women TONY SCARIA 2010 KMC • Hip  rare • More common in older men • Hand • More commonly involved in elderly female • DIP PIP • Non nodal erosive IP arthritis • Carpo metacarpal joint of thumb TONY SCARIA 2010 KMC
  • 67.
    11-03-2018 • OA characteristicallyinvolves • DIP  Heberdens node *** • PIP  Bouchards node • H is more distal than B in alphabets • Base of thumb 1st MCP • Other MCP & wrist are spared TONY SCARIA 2010 KMC • Foot • Hallux valgus/rigidus • Hammer toes TONY SCARIA 2010 KMC
  • 68.
    11-03-2018 TONY SCARIA 2010 KMC Radiologicalfeatures of OA • 'asymmetric narrowing of joint space‘ • Due to wearing of cartilage • EARLIEST SIGN • sclerosis of the subchondral bone under the area of cartilage loss, • d/t new bone formation • cysts close to the joint surface and • osteophytes at the margins of the joint • Loose bodies & deformities TONY SCARIA 2010 KMC
  • 69.
    11-03-2018 • In kneejoint  medial joint space is affected first  genu varus TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 70.
    11-03-2018 Osteo arthritis -Womack grading. • Stage i • there is softening of the cartilage if the surfaces of the knee. This may not show on X-ray. • Stage ii • the cartilage starts to wear away and shows as a narrowed joint space. • Stage iii • osteophytes are seen. Stage 2 and 3 may merge and be difficult to separate. • Stage iv • "bone-on-bone" with very narrow joint space, lots of osteophytes, and distortion of the joint TONY SCARIA 2010 KMC Osteo arthritis - Outerbridge  cartilage Classification • Grade 0: normal cartilage; • Grade I: cartilage with softening and swelling; • Grade II: a partial-thickness defect with fissures on the surface that do not reach subchondral bone or exceed 1.5 cm in diameter; • Grade III: fissuring to the level of subchondral bone in an area with a diameter more than 1.5 cm; • Grade IV, exposed subchondral bone TONY SCARIA 2010 KMC
  • 71.
    11-03-2018 Rx • i. Initialmanagement of • 1. Physical therapy • 2. Bracing, orthoses, ambulatory aids • 3. NSAID, glucosamine, chondroitin, intraarticular injections of steroid or hyaluronic acid, and analgesics. TONY SCARIA 2010 KMC • Glucosamine • Increases lubrication • Chondroitin sulphate • Increases lubrication • Diacerin • Reduces IL -1 induced cartilage damage • S – adenosyl methionine(SAM) • Reduces pain & cartilage damage • Tetracycline • Inhibits metalloproteinase • Not routinely used TONY SCARIA 2010 KMC
  • 72.
    11-03-2018 • Sx • Insevere disabling pain • Interfering with day to day activities • Grade 2- 5 radiological evidence TONY SCARIA 2010 KMC • ii. Arthroscopic débridement • active, older adults with mild-to-moderate OA of the knee after conservative treatment • Only in presence of mechanical symptom like locking / loose bodies in X ray • iii. High tibial osteotomy • It is a well-established procedure for the treatment of unicompartmental OA of the knee. • To decrease weight distribution in that compartment TONY SCARIA 2010 KMC
  • 73.
    11-03-2018 TONY SCARIA 2010 KMC •Type I to III conservative management • Non pharmacological • Life style modification /physiotherapy/bracing • pharmacological • NSAIDS/intrarticular injection of steroid • Type IV & V  surgery • Arthroscopic wash out / debridement • Realignment Sx • High tibial osteotomy • Unicompartmental arthroplasty • TKR TONY SCARIA 2010 KMC
  • 74.
  • 75.
    11-03-2018 Indications for arthroplasty Totalknee replacememt • Pain in severe OA • Deformities • Severe pain from chondrocalcinosis & pseudogout & severe patellofemoral OA • Tricompartmental OA Total hip replacement • Alleviation of pain • Improve function of hip • Arthritis • AVN • Bone tumours involving acetabulum & proximal femur TONY SCARIA 2010 KMC Rheumatoid arthritis • c/c immunologically mediated • Persistent inflammatrory synovitis • Symmetrically involving peripheral joints • Cartilage damage /bone erosion /change in joint integrityTONY SCARIA 2010 KMC
  • 76.
    11-03-2018 • Pathogenesis • Women(30-50 yrs) • HLA DR4 DW4 • Initial site  synovial membrane TONY SCARIA 2010 KMC Stages • Initiation phase • d/t non specific inflmn • Amplification phase • d/t T cell activation • c/c inflammatory phase • Due to cytokines IL- 1 , TNF alpha & IL - 6 TONY SCARIA 2010 KMC
  • 77.
  • 78.
    11-03-2018 TONY SCARIA 2010 KMC •Score of > 6 = definite RA TONY SCARIA 2010 KMC
  • 79.
  • 80.
    11-03-2018 Pathogenesis of RA TONYSCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 81.
    11-03-2018 TONY SCARIA 2010 KMC Manifestationsof RA • Erosive arthritis • Joint swelling tenderness & limitation of motion • Pain aggravated by movement  Mc manifestation • Axial involvement • Limited to Atlantoaxial joint (cervical spine) • Bakers cyst • Extension of inflamed synovium in to popliteal space TONY SCARIA 2010 KMC
  • 82.
    11-03-2018 Involvement of hand •Z deformity • Radial deviation of wrist • Ulnar deviation of fingers of hand *** • pathognomonic TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 83.
    11-03-2018 At DIP malletdeformity Rupture of extensor tendon at DIP TONY SCARIA 2010 KMC Swan neck deformity • Extension of PIP & compensatory flexion of DIP TONY SCARIA 2010 KMC
  • 84.
    11-03-2018 boutonniere deformity • Flexionof PIP & extension of DIP TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 85.
    11-03-2018 Intrinsic plus hand deformityin RA (d/t contracture of lumbricals) Assessed by bunnels test TONY SCARIA 2010 KMC • Eversion of hind foot deformity • @ subtalar joint • plantar subluxation of metatarsal head • Hammer toes • Widening of forefoot • Claw toe TONY SCARIA 2010 KMC
  • 86.
    11-03-2018 Windswept deformity • Valgusdeformity of toes in foot & varus in other TONY SCARIA 2010 KMC Hammer toe / contracted toe • Deformity of 2nd 3rd 4th toe • Causing it to be permanently bent • Mallet toe • Similar condition affecting DIP TONY SCARIA 2010 KMC
  • 87.
    11-03-2018 Claw toe • Dorsiflexionof proximal phalanx on lesser MTP joint • Combined with flexion of DIP & PIP TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 88.
    11-03-2018 Rheumatoid arthritis ofspine • Involves usually atlantoaxial joint • Dens erosion through foramen magnum • May encroach spine TONY SCARIA 2010 KMC Knee joint in RA • Valgus • Triple deformity • Flexion + • Posterior subluxn + • External rotation TONY SCARIA 2010 KMC
  • 89.
    11-03-2018 Rheumatoid nodules • inpressure areas such as the elbow, finger joints, Achilles tendon and occipital part of scalp TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 90.
    11-03-2018 rheumatoid vasculitis • Polyneuropathy •Mononeuritis multiplex • Digital gangrene • MI TONY SCARIA 2010 KMC Skin involvement in RA • Vasculitis  skin necrosis • Malignant rheumatoid arthritis TONY SCARIA 2010 KMC
  • 91.
    11-03-2018 Eye – • a.Episcleritis • b. Scleritis • c. Conjunctivitis • d. Scleromalacia (Rupture of the Globe) can occurs Uveitis is not a feature TONY SCARIA 2010 KMC Neurological symptoms – • 1. Peripheral neuropathy – due to vasculitis. • 2. Cord compression – due to atlanto axial subluxation • 3. Nerve entrapment – due to proliferative, synovitis • (Carpal tunnel syndrome due to median nerve compression, tarsaltunnel syndrome due to Posterior tibial nerve compression). TONY SCARIA 2010 KMC
  • 92.
    11-03-2018 Pleuropulmonary manifestation ofRA • Pleuritis • Pleural effusion • Mainly on left side • Low pleural fluid sugar • Arteritis • Pneumonitis • Pleuropulmonary nodule TONY SCARIA 2010 KMC Caplans syndrome • Pneumoconiosis • RA • Lung nodules TONY SCARIA 2010 KMC
  • 93.
    11-03-2018 Multiple Lung nodules are alsofound in Caplan’s syndrome Wegeners granulomatosis Silicosis TONY SCARIA 2010 KMC Pericarditis • Constrictive pericarditis • Asymptomatic TONY SCARIA 2010 KMC
  • 94.
    11-03-2018 • RENAL INVOLVEMENTIS NOT A FEATURE OF RA • EVEN IF OCCURS D/T AMYLOIDOSIS OR DRUG INDUCED TONY SCARIA 2010 KMC Radiologic changes in RA • Soft tissue swelling • Joint effusion • Symmetrical involvement • Juxta aticular osteopenia • With in weeks • Loss of articular cartilage & erosion • Narrowing of joint space • Subluxation & ankyloses • Lack of hypertrophic changes • Like sclerosis / osteophyte TONY SCARIA 2010 KMC
  • 95.
    11-03-2018 Rheumatoid factor • Isused in diagnosis & prognosis • Not used for screening • About 80% of individuals with rheumatoid arthritis have autoantibodies to the Fc portion of autologous IgG (rheumatoid factors). • These are mostly IgM antibodies • RF & Ig combine to form d/s process • correlates with ds activity • decline in the level of rheumatoid factor is associated with reduced clinical disease activity TONY SCARIA 2010 KMC • RF is also positive in • 5% healthy people • SLE • c/c liver ds • Sjogrens syndrome • Pulm fibrosis • IMN • Hep B • TB • Leprosy • Syphilis • SABE • Leishmaniasis • Malaria • schistosomiasis TONY SCARIA 2010 KMC
  • 96.
    11-03-2018 Diagnosis • Normocytic normochromicanemia • Raised ESR CRP & ceruloplasmin • Anti CCP Is positive in RA • Most specific Ab • Synovial fluid analysis • Turbid • Decreased viscosity • Increased protein content • Decreased sugar • WBC 5000- 50000/ml TONY SCARIA 2010 KMC Marker of active ds • Anemia of c/c ds • Increased ESR CRP & platelet count • Decreased synovial fluid viscosity TONY SCARIA 2010 KMC
  • 97.
    11-03-2018 Feltys syndrome TONY SCARIA 2010KMC Feltys syndrome • Complement level is decreased • • Cause of leukopenia in RA is • 1. Drug induce – most common • 2. Felty’s syndrome • 3. T-LGL TONY SCARIA 2010 KMC
  • 98.
    11-03-2018 Causes of leukopenia •Feltys syndrome • Drug induced  most common • T- IGL TONY SCARIA 2010 KMC Haematology in RA • Anemia in RA Normocytic normochromia • d/t ineffective erythropoiesis • GI bleed a/w NSAIDs • eosinophilia in RA • Severe systemic ds • Large B cell lymphoma  lymphoma in RA TONY SCARIA 2010 KMC
  • 99.
    11-03-2018 Eye • Scleritis • Episcleritis •Conjunctivitis • Scleromalacia (rupture of globe) • Uveitis is not a feature TONY SCARIA 2010 KMC Neurological system • CNS is not directly involved • Peripheral neuropathy • d/t vasculitis • Cord ompression • d/t atlanto-axial subluxation • Nerve entrapment • d/t proliferative + synovitis  carpel tunnel syndrome • Tarsal tunnel syndrome • Posterior tibial nerve compression TONY SCARIA 2010 KMC
  • 100.
    11-03-2018 Poor prognosis • Hightitres of RF • C-RP • Haptoglobulin • Subcutaneous nodules • Radiographic evidence • Life expectancy is decreased by 3-7 yrs TONY SCARIA 2010 KMC Complications of RA • Amyloidosis  NS • Septic arthritis • By staph aureus TONY SCARIA 2010 KMC
  • 101.
    11-03-2018 • NSAIDs • Etodolac chiral NSAID for Rx of RA • Licofelone • Inhibits both lipoxygenase & cyclooxygenase of AA metabolism • No GI s/e • Analgesic anti inflammatory /antihistaminic • Flupirtine • Selective K+ channel opener • NMDA receptor antagonist properties • Non NSAID non opid nonsteroidal analgesic TONY SCARIA 2010 KMC DMARDs • Slow down progression of ds • Methotrexate *** • Mc used • D – pencillamine • Sulfasalazine • Gold • HCQ TONY SCARIA 2010 KMC
  • 102.
    11-03-2018 TONY SCARIA 2010 KMC s/eof DMARDs • Gold/pencillamine • NS • Pancytopenia • HCQ • Retinopathy • Sulphasalazine • Skin rash • Methotrexate • Liver dysfunction TONY SCARIA 2010 KMC
  • 103.
    11-03-2018 Glucocorticoid rx inRA • Vasculitis • Mononeuritis multiplex • Pericarditis • Perforating eye lesion • Pleural effusion TONY SCARIA 2010 KMC Anti TNF alpha drugs • Adalimumab • Certrolizumab • Etanarcept • Golimumab • infliximab TONY SCARIA 2010 KMC
  • 104.
    11-03-2018 s/e anti TNFalpha drugs • Serious infections • Disseminated TB • Pancytopenia • Demyelination disorders • Hypersensitivity • Exacerbation of CHF • Hypersensitivity • Severe liver disease • SLE • NO INCREASED RISK OF MALIGANANCY • Avoided in patients with hypersensitivity / active infection TONY SCARIA 2010 KMC Tocilizumab • Monoclonal Ab directed against IL – 6 receptor cell membrane • IL – 6  involved in pathogenesis of RA  joint inflammation & damage TONY SCARIA 2010 KMC
  • 105.
    11-03-2018 IL-1 receptor antagonist •ANAKINRA TONY SCARIA 2010 KMC Rituximab • Anti CD – 20 Ab • Monoclonal Ab • Attaches to B cell TONY SCARIA 2010 KMC
  • 106.
    11-03-2018 ibritumomab tiuxetan • Murinemonoclonal Ab labelled with yttrium 90 or indium 111 • In cases refractory to rituximab TONY SCARIA 2010 KMC Abatacept  fusion protein TONY SCARIA 2010 KMC
  • 107.
    11-03-2018 TONY SCARIA 2010 KMC Trastuzumab •Anti – HER2 /neu Ab • Monoclonal Ab directed against HER2 /neu receptor tyrosine kinase against breast cancer with over expression of this factor TONY SCARIA 2010 KMC
  • 108.
    11-03-2018 Immunosuppressants • Cyclophosphamide • Leflunomide •Azathioprine • Cyclosporine TONY SCARIA 2010 KMC Orthopedic procedures in patients with RA • Tenosynovectomy • Tendon repair • Synovectomy • arthroplasty • Arthrodesis TONY SCARIA 2010 KMC
  • 109.
    11-03-2018 • 1. Anemia– *Normocytic normochromic anemia* and is due to ineffective • erythropoiesis • 2. ESR – It is increased in all patients with active RA. • 3. C – reactive protein and ceruloplasmin are increased • 4. Synovial fluid analysis – • 1. Turbid • 2. Decreased viscosity , • 3. Increase protein content • 4. Decrease glucose concentration • 5. WBC count is 5000 – 50000 per ml TONY SCARIA 2010 KMC Psoriatric arthritis TONY SCARIA 2010 KMC
  • 110.
    11-03-2018 Psoriatic arthritis • Seronegativepolysynovitis erosive • HLA DR7 • HLA CW6 • Mc presentation  assymmetric oligoarthritis of large joints • High incidence of sacroilitis & spondylitis • HLA B27 • RF negative TONY SCARIA 2010 KMC Psoriatic arthritis • In 30% psoriatic patients • Skin lesions (early) joint involvement (late) • Nail changes+ • PsA features include • DIP joint involvement • Dactylitis (sausage digitals) • Arthritis mutilans(severe destructive arthritis) • Corneal nodules • Liver damage • Rx • MTX ,etanarcept TONY SCARIA 2010 KMC
  • 111.
    11-03-2018 CF • 5 patternsof joint involvement • Arthritis of DIP joint • Asymmetrical oligoarthritis  MC • Symmetrical poly arthritis similar to RA • Axial involvement  spine & sacroiliac joint • Arthritis mutilans TONY SCARIA 2010 KMC • Shortening of digits (d/t underlying osteolysis)TELESCOPING • Pencil in cup deformity + TONY SCARIA 2010 KMC
  • 112.
    11-03-2018 • 1. DIPinvolvement, & classical pencil in cup deformity • 2. Marginal erosion with adjacent bony proliferation, (whiskering) • 3. Small joint ankylosis • 4. Osteolysis of phalangeal and metacarpal bone, with telescoping of digits • 5. Periostitis and proliferative new bone at sites of enthesitis • 6. Severe cervical spine involvement with atlantoaxial subluxation, but relative sparing of thoracolumbar spine, and paravertebral ossification. TONY SCARIA 2010 KMC Pencil in cup deformity TONY SCARIA 2010 KMC
  • 113.
    11-03-2018 Pencil in cupdeformity TONY SCARIA 2010 KMC Whiskering + TONY SCARIA 2010 KMC
  • 114.
    11-03-2018 Saucage digitals d/tdactylitis TONY SCARIA 2010 KMC arthritis mutilans TONY SCARIA 2010 KMC
  • 115.
    11-03-2018 • Telescoping • Underlyingosteolysis  telescoping • Nail changes • Less tender than RA • Conjunctivitis & Ch uveitis • Fibrous & bony ankylosis • PsA>> RA TONY SCARIA 2010 KMC Radiological signs • Opera glass hand (telescoping) • Telescoping of bones in to its neighbours with shortening of digits • Pencil in cup sign • Tapering of proximal phalanx and bony prolifern of distal phalanx TONY SCARIA 2010 KMC
  • 116.
    11-03-2018 Radiological signs inPsA • Whiskering • Marginal erosions with adjacent bony proliferation • Mouse ear TONY SCARIA 2010 KMC Opera glass hand sign TONY SCARIA 2010 KMC
  • 117.
    11-03-2018 TONY SCARIA 2010 KMC Criteriafor PsA caspar TONY SCARIA 2010 KMC
  • 118.
    11-03-2018 Rx of PsA •Methotrexate is drug of choice • Anti TNF - a agents • etanercept and infliximab are newer drugs and are effective even in longstanding resistent PsA cases (to previous therapy) and extensive skin lesion • Other effective agents are sulfasalazine, cyclosporine retinoic acid & psoralen & UV-A (PUVA) TONY SCARIA 2010 KMC Seronegative spondyloarthropathies • Onset <40 years • HLA B 27 positive • Presence of uveitis • Absence of auto Ab • Inflammatory of arthritis of spine / large joints TONY SCARIA 2010 KMC
  • 119.
  • 120.
    11-03-2018 Ankylosing spondylitis /marie – strumpell/bechtew ds • Genetically determined • Generalised c/c inflammatory ds • Primarily affects axial skeleton • With variable involvement of shoulder or hip TONY SCARIA 2010 KMC • Primary pathology • Enthesitis • Site of ligamentous attachment to bone • Primarily I pelvis & spine • Prominent edema of adjacent bonemarrow fb ossification • Peripheral arthritis of AS • TNF alpha & cytokines TONY SCARIA 2010 KMC
  • 121.
    11-03-2018 • AS • HLAB27 +ve • M:F=3:1 • Earliest manifestation  sacroilitis (enthesitis+ synovitis) • Sacroiliac joint mainly on iliac side • Extra articular manifestation • a/c anterior uveitis *** mc • cardiac manifestation  AR , 3rd degree heart block , conductive defects • GI involvement  inflammation of colon / ileum (IBD) • Restrictive lung ds TONY SCARIA 2010 KMC • M:F=2:1 • 15 – 25 yrs • dull pain, insidious in onset, felt deep in lower lumbar or gluteal region, accompanied by low back morning stiffness of up to few hour duration that improves with activity and returns following period of inactivity TONY SCARIA 2010 KMC
  • 122.
    11-03-2018 Musculoskeletal system • Enthesopathy •Inflammation of tendon & ligament attached to it • Synovitis of diarthroidal joints • Sacroiliac joint • Facet foint of vertebrae • Costovertebral joint • Inflammation of fibroosseous junction of syndesmotic joints • SI joint ligaments • Manubrium sternii • Pubic symphysis • Intervertebral disc TONY SCARIA 2010 KMC 1. ? Mark posture • Hyperkyphosis of thoracic spine & loss of lumbar lordosis 2. Sacroilitis TONY SCARIA 2010 KMC
  • 123.
    11-03-2018 TONY SCARIA 2010 KMC •Should increase by atleast 5 cm in on fexion • <5cm  restriction of flexion TONY SCARIA 2010 KMC
  • 124.
  • 125.
    11-03-2018 TONY SCARIA 2010 KMC Limitationof chest expansion • d/t costovertebral joint involvement • <2cm  AS TONY SCARIA 2010 KMC
  • 126.
    11-03-2018 Eye involvement inAS • Iritis + TONY SCARIA 2010 KMC Spine • Loss of lumbar lordosis (Straightening) • Enthesitis – Increased Blood flow – Cause absorption by • Erosions of anterior corners of vertebral body causing squaring of vertebral body. • Delicate syndesmophyte with “vertical orientation bridging vertebral bodies” “(horizontal in degenerative spine disease)”. • Bamboo spine, syndesmophytes and paravertebral ossifcation TONY SCARIA 2010 KMC
  • 127.
    11-03-2018 • Xray • Lossof lumbar lordosis • Sclerosis of anterior corners of vertenbral body • Squarring of vertebrae • Bamboo spine TONY SCARIA 2010 KMC Sacro iliac joint • Blurring of margins • Juxta-articular sclerosis • Erosions cause pseudowidening • Obliteration of joint (fbrous followed by bony ankylosis) TONY SCARIA 2010 KMC
  • 128.
    11-03-2018 • Complication • Mc spine # even with minor trauma TONY SCARIA 2010 KMC • Rx • Phenylbutazone  most effective drug • Causes aplastic anemia • Hence reserved for resistant cases • indomethacin • Sulfasalazine + FA antagonist • Anti TNF alpha therapy • Infliximab / etanarcept TONY SCARIA 2010 KMC
  • 129.
    11-03-2018 Diffuse idiopathic skeletal hypertrostosis(DISH) Ankylosing spondylitis Syndesmophytes Nonmarginal (paravertebral) Marginal X ray Flowing candle wax • Bamboo spine • Squaring of vertebral bodies Radiodensity Increased Radiolucent HLA B8 B27 a/w DM + - Age Elderly Young TONY SCARIA 2010 KMC Flowing wax like appearance in DISH TONY SCARIA 2010 KMC
  • 130.
    11-03-2018 TONY SCARIA 2010 KMC Neuropathicjoint d/s / charcot’s joint • Progressive destructive arthritis • d/t loss of sensation & proprioception  repeated trauma  bone & cartilage damage • Hence Less pain is experienced than anticipated • DM ***>>tabes dorsalis • Radiological features • Same as OA • Diagnosis • MRI & bone scan • Using indium 111 labelled WBC / IgG (to differentiate from OM increased uptake in osteomyelitis but not in neuropathic joint) TONY SCARIA 2010 KMC
  • 131.
    11-03-2018 d/s associated withcharcots joint • DM*** • Midtarsal >>tarsometatarsal>>metatarsophalangeal>>ankle >> knee & spine • Tabes dorsalis • Associated with lightening pin in lower limbs • Knee joint is affected • Meningomyelocele • Ankle / intertarsal • Peroneal muscular atrophy • Syringomyelia • Shoulder elbow wrist cervical spine • Leprosy • Amyloidosis • c/c alcoholism • Metatrsophalangeal / IP TONY SCARIA 2010 KMC Radiological finding in charcots joint • Joint space narrowing • Joint becomes progressively enlarged from bony overgrowth and synovial effusion • swelling, instability, laxity & progressive deformity TONY SCARIA 2010 KMC
  • 132.
    11-03-2018 Tumbling building blockspine • In charcot spine TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 133.
    11-03-2018 Rx of charcotsjoint • limitation of joint movements by bracing or casting, joint debridement (arthrocentesis) and fusion of joint (arthrodesis) TONY SCARIA 2010 KMC Hemophilic arthropathy • Order of joint involvement • Knee >> ankle >> elbow>> shoulder>>hip • Position of joint • Flexion + abduction + external rotation • Avoid joint aspiration & arthroscopy TONY SCARIA 2010 KMC
  • 134.
    11-03-2018 • Hemophilia • X-linkedrecessive disorder • manifested only in males but females are carriers • characterized by the absence or • defciency of factor VIII (hemophilia A or classical hemophilia)  85% • factor IX (hemophilia B or Christmas disease) TONY SCARIA 2010 KMC Haemarthrosis • Ankle is most commonly involved in children • Joint assumes position of minimal discomfort and minimal intra-articular pressure, which is, • flexion (30–65°) abduction (15°) and lateral rotation (15°) in hip; and slight flexion in knee. • Pain, warmth, boggy swelling, tenderness and limited movements cause it to resemble with low grade infection. TONY SCARIA 2010 KMC
  • 135.
    11-03-2018 • Clotting Factors •• <1% ~ Spontaneous Hemorrhage • • 1-5% - Hemorrhage on Mild Trauma • • >5% Hemorrhage on Signifcant Trauma TONY SCARIA 2010 KMC Haemophilic pseudotumour • In severe haemophiliacs • Progressive cystic swelling caused by uncontrolled haemorrhage with in confined space • Haematoma increases in size  pressure necrosis • Mc location  thigh >> abdomen > pelvis • Iliopsoas >>> quadriceps TONY SCARIA 2010 KMC
  • 136.
    11-03-2018 Intramuscular bleeding • Bleedinginto muscle is less common but equally harmful can cause muscle necrosis, reactive fbrosis and joint contractures. • Mc site of bleeding in LL • Quadriceps • Mc site of bleeding in UL • Deltoid • Bleeding into iliopsoas & retroperitoneum • Resembles APPENDICITIS TONY SCARIA 2010 KMC Radiologic features of haemophilic arthritis • Soft tissue swelling • Juxtaarticular osteopenia (not sclerosis) • Overgrowth & osteoporosis of epiphyses • Marginal erosions • Subchondral cyst • Widened intercondylar notch of femur • Trochlear notch of ulna • Enlarged proximal radius • Squarring distal end of patella • Total loss of joint space • Fibrous ankylosis TONY SCARIA 2010 KMC
  • 137.
    11-03-2018 Rx • Factor replacementin haemophilia • Joint aspiration (in severe cases) • Ice & analgesics  to relieve pain • Rest • Compressive bandage TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 138.
    11-03-2018 TONY SCARIA 2010 KMC Gout •1st MTP  mc involved joint • Monosodium crystals  • negatively birefringent • Needle shaped • Tophi • Extensor aspect of elbow • Pinna • 1st MTP TONY SCARIA 2010 KMC
  • 139.
  • 140.
  • 141.
    11-03-2018 Acute gout • Firstepisode @ night • With dramatic joint pain & swelling • Skin is shiny red  similar to cellulitis TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 142.
    11-03-2018 TOPHI in Xray TONYSCARIA 2010 KMC MARTELS SIGN or G (GOUT) SIGN • Punched out with over hanging bone • Chronic tophaceous gout • -Cystic changes and well-defined erosions described as punched-out lytic lesions with overhanging bony edges (Martels sign, or G sign for gout), associated with soft tissue calcified masses TONY SCARIA 2010 KMC
  • 143.
    11-03-2018 MSU crystals inpolarised light TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 144.
  • 145.
  • 146.
    11-03-2018 Pseudogout • Calcium pyrophosphatecrystals • Rhomboid /rectangular • Positively refringent • Moderate pain • Large joints  knee joint – mc involved joint • Chondrocalcinosis on xray TONY SCARIA 2010 KMC Pseudogout is a/w • Hypothyroidism • Hemochromatosis • Hyperaprathyroidism (primary) • Hypomagensemia • Hypophosphatemia • Wilsons ds TONY SCARIA 2010 KMC
  • 147.
    11-03-2018 • Chondrocalcinosis • Radiologicalhall mark of pseudogout • Linear /multiple punactate in menisci TONY SCARIA 2010 KMC TONY SCARIA 2010 KMC
  • 148.
  • 149.
    11-03-2018 Milwaukee shoulder • Morecommon in female • With h/o trauma • d/t deposition of calcium hydroxyapatite crystal • Mc in shoulder • Definitive diagnosis of apatite arthropathy depends on identification of crystals from synovial fluid or tissue • X ray  multiple loose bodies • Rx • Intraarticular steroid TONY SCARIA 2010 KMC Reiter syndrome • Reactive arthritis • Seronegative spondyloarthropathies • HLA B27 + • Classic triad • Conjunctivitis / uveitis • Arthritis large joints in LL • Urethritis • Circinate balanitis + • Keratoderma blenorrhagica TONY SCARIA 2010 KMC
  • 150.
    11-03-2018 • Bacteria causingReactive Arthritis • 1. Salmonella • 2. Shigella MC ORGANISM • 3. Campylobacter • 4. Yersinia • 5. Chlamydia. MC cause following urethritis • Reiter disease can occur in epidemic form TONY SCARIA 2010 KMC Rx of reiters ds • 1. Analgesics • 2. Local corticosteroids are useful in the case of iritis • 3. The nonspecific urethritis is treated with a short course of • Tetracycline. TONY SCARIA 2010 KMC
  • 151.
    11-03-2018 Ankylosis Bony ankylosis • Pyogenicarthritis of all joints *** mc cause • Tuberculous arthritis of spine Fibrous ankylosis • Tuberculous arthrotis of hip / knee /small joints TONY SCARIA 2010 KMC Transient synovitis of hip • aka Observation hip • Self limiting condition usually lasts only a short time • aka Coxalgia fugax /coxitis serosa • Mc of hip pain in children < 10 yrs (3-12 yrs of age) • With rapid onset of pain • Limited to groin or may be referred to thigh • limping • Antalgic gait • limited range of movement • Gaurded rotation of hip • Tenderness at extremes of motion • Following viral URTI • Usually u/l TONY SCARIA 2010 KMC
  • 152.
    11-03-2018 FABER  flexionabduction external rotation TONY SCARIA 2010 KMC X ray • Widened joint space medially • Bone density is normal TONY SCARIA 2010 KMC
  • 153.
    11-03-2018 USG • Mild jointeffusion • USG guided aspiration • For definitive cytological diagnosis • To r/o septic arthritis TONY SCARIA 2010 KMC Rx • Observation • Traction TONY SCARIA 2010 KMC
  • 154.
    11-03-2018 Chondrocalcinosis • Knee joint MC involved in menisci • Causes • All causes Pseudogout  most important cause • Alkaptonuria • Wilsons ds TONY SCARIA 2010 KMC Arthrtis Erosive arthritis • RA • Gout • Hyperparathyroidiam Nonerosive • SLE • OA TONY SCARIA 2010 KMC
  • 155.
    11-03-2018 Spondyloarthrosis • Aka Seronegativearthritis • 1. Ankylosing spondylitis • 2. Reiter’s disease • 3. Reactive Arthritis • 4. Psoriatic arthritis • 5. Juvenile rheumatoid arthritis (JRA) • 6. Inflammatory bowel disease TONY SCARIA 2010 KMC Features of spondyloarthrosis • 1. Seronegativity (rheumatoid factor –ve) • 2. HLA B27 associated • 3. Axial arthritis: Pathology in spine and sacroiliitis. • 4. Asymmetrical large-joint oligoarthritis (i.e <5 joints) or monoarthritis. • 5. Enthesitis: Inflammation of the site of insertion of tendon or ligament into bone, eg. Plantar fasciitis, Achillis tendonitis, costochondritis. • 6. Extra-articular manifestations eg anterior uveitis, aortic regurgitation TONY SCARIA 2010 KMC
  • 156.
    11-03-2018 Arthritis in children TONYSCARIA 2010 KMC Acute(<2 weeks ) •Rheumatic fever •Septic arthritis Sub acute(2-6 weeks) •Reactive arthritis Chronic(>6 weeks) •JRATONY SCARIA 2010 KMC
  • 157.
    11-03-2018 JIA Juvenile rheumatoid arthritis Juvenile psoriatic arthritis Juvenileenthesitis related arthritis TONY SCARIA 2010 KMC Juvenile rheumatoid arthritis • Diagnostic criteria TONY SCARIA 2010 KMC
  • 158.
    11-03-2018 JRA pauciarticular Type I Type II Polyarticular Polyarticular RApositive Polyartciular RA negative Systemic onset • ANA +ve • RF –ve • Eye involvement with iridocyclitis glaucoma cataract • HLA DR5 & DRW8 • ANA –ve • RF-ve • Iritis only (not iridocyclitis) • HLA B27 • ANA –ve • RF-ve • Iritis only (not iridocyclitis) • HLA B27 • Symmetrical joint involvement with uveitis & rheumatoid nodule • RA factor + • ANA +ve • No rheumatoid nodule • No RF • Fever with ras • lymphadenopathy HSM • RF –ve • Uveitis (iridocyclitis ) absent TONY SCARIA 2010 KMC RF is positive only in polyarticular JRA TONY SCARIA 2010 KMC
  • 159.
    11-03-2018 • No iritisor uveitis in systemic type & polyarticular RA negative TONY SCARIA 2010 KMC Rx • NSAIDs • Naproxen • Ibuprofen • Indomethacin TONY SCARIA 2010 KMC