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  1. 1. Martin Edobor, John Lewin Rheumatology Crash Course
  2. 2. Objectives • Go through GALS screen OSCE station • Cover key Rheum Topics • Practice SBA • General Advice
  3. 3. OSCE
  4. 4. Gals screen - Introduction 1. Introduces self and states role 2. Consent and obtains patients name 3. Offers a chaperone 4. Exposure of patient (giving them privacy to do so) 5. Asks the following questions: What is your occupation? Do you have any pain or stiffness in any joints, muscles or your back? Do you have any difficulty climbing the stairs? Do you have any difficulty washing or dressing? 6. General inspection for obvious scars, deformities, abnormal posture or muscle wasting
  5. 5. 7. GAIT: asks patient to walk away and turn and come back, checking for: Smoothness of movement, abnormalities in gait, normal heel toe strike, normal‐ turning speed, normal posture and arm swing Gals screen - Gait
  6. 6. Gals screen – Spine (1) SPINE: inspect from the back, looking for: Any deformities such as scoliosis Level shoulders and iliac crests Normal cervical and lumbar lordosis 9. Checks muscle bulk in the paraspinals, shoulder girdle, gluteals, calves
  7. 7. 10. Inspects the popliteal fossa and Achilles tendon 11. Feels supraspinatus and lightly squeezes trapezius to test for fibromyalgia 12. Runs finger down back to check for tenderness 13. Movements: Side to side, rotate upper torso without moving hips‐ ‐ Press ear to shoulder Open jaw and move side to side‐ ‐ Flex and extend neck, rotate head Gals screen – Spine (2)
  8. 8. ARMS: General inspection, comment on deformity or erythema over the joints, Dupuytren’s or thenar wasting in the hand, pitting psoriasis in the nails 16. Feels the temperature of the joints then squeezes across the metacarpals for tenderness 17. Movements:  Put hands behind head and push elbows back, touch the small of back, ‘pray’ with hands together then back to back  Flex and extend elbows, pronation and supination, flex and extend wrists 18. Hands:  Power grip  Precision movements (thumb to each finger in turn) Gals screen - Arms
  9. 9. LEGS: General inspection for muscle wasting, fasciculations, skin rashes, nodules, swelling, deformity or erythema over the joints, calluses or ulcers on soles 20. Feels the temperature of the joints then squeezes across the metatarsals for tenderness 21. Movements:  Flex and extend hips, knees and ankles  Internal rotation at hips and knees  Inversion and eversion at the ankle  Crepitus and bulge test at the knee Gals screen - Legs
  10. 10. 22. Thanks patient, offers to help them dress 23. To complete my examination, would like to do a full neurological examination of the upper and lower limbs and a full cranial nerve exam 24. Presents findings in a clear and fluent manner, for example “This patient doesn’t have any pain or stiffness in any of his joints, muscles or back. He has no difficulty climbing stairs or washing and dressing. On general inspection, there were no obvious scars, deformities, abnormal posture or muscle wasting. On examination GAIT was smooth with no abnormalities. He displayed no weakness and normal range of movement in arms, leg and spine. This patient has a normal GALS screen” Gals screen - Closing
  11. 11. Rheumatoid Arthiritis (1) A chronic symmetrical inflammatory polyarthititis with systemic involvement Epidemiology: F>M 3:1, ages 30-50 Associated with HLA-DR4 Pathology: Autoantibodies (IgM) to Fc of IgG, forming immune complexes which deposit in joints leading to inflamation. Symptoms: Pain (decrease with activity), swelling, morning stiffness >30mins,. Joint Involvement: MCP, MIP, PIP, MTP SYMETRICAL!
  12. 12. Rheumatoid Arthritis (4) Systemic extra-articular involvement: Skin: Rhumatoid nodules, synivotendonitis Lungs: Effusions, nodules, fibroisis, Caplans syndrome: a combination of pneumoconiosis and RA Eyes: Sclertis (painful red eye), Episcleritis(non-painful red eye), Sorgjerns syndrome Neural: Mononeuritis multiplex, Carpal tunnel Felty Syndrome: RA with splenomegaly and neutropenia Normochromic normocytic anaemia
  13. 13. Rheumatoid Arthritis (5) Investigations ESR, CRP Increased Possible Anaemia RF +ve in 70% of cases, Anti-ccp positive in >90% of cases CXR –Swelling of soft tissue, erosions, joint narrowing, juxto-articular osteopenia
  14. 14. Rheumatoid Arthritis (6) Management 2009 NICE guidelines it is recommend that patients with newly diagnosed active RA start a combination of DMARDs (including methotrexate and at least one other DMARD, plus short-term glucocorticoids) 1.DMARDS Methotrexate (s.e derange LFT’s renal, neutopenia, PF, teratogenic) •Sulfasalazine (s.e infertility, G6PD heamolysis) •Lefunimide Others e.g Azathriopine, Gold (Also add prednisolone)
  15. 15. The current indication for a TNF-inhibitor is an inadequate response to at least two DMARDs including methotrexate 4. Anti TNF alpha, use if DMARDS failed e.g Entgeracept, Adelumimab , Infliximab Side effects increase risk of cancer, infection, reversible refulx and demylinating disease. If a diagnosis of RA is yet to be made, you can give your patient NSAIDS (if no contraindication) or a steroid injection
  16. 16. A 47 year old woman presents to A+E with dyspnoea. An X-ray confirms a large right sided pleural effusion. Fluid is then aspirated from the right lung. Clinical chemistry analysis showed a pleural fluid lactate dehydrogenase (LDH): serum LDH ratio of 0.76. Which of the following is the most likely cause of this patient’s pleural effusion? • Meigs’ syndrome • Hypothyroidism • Constrictive pericarditis • Nephrotic syndrome • Rheumatoid arthritis
  17. 17. Pleural effusions • Transudate – Increased venous pressure – Hypoproteinaemia – Hypothyroidism – Meigs syndrome • Exudate – Increased capillary perm 2ndry to cytokines • Infection • Inflammation • Malignancy
  18. 18. SBA question • A 53 year old woman with stable rheumatoid arthritis is to undergo an elective cholecystectomy. Arthropathy is the only finding on clincial examination. Which of the following is the most important investigation prior to the procedure? • A) Chest X-ray • B) Echocardiography • C) ABG • D) Cervical spine imaging • E) Uric acid levels
  19. 19. ANSWER • C) 1 in 3 patients with RA have atlanto-axial subluxation. • Hyperextension of C-spine during intubation risks cervical myelopathy • Imaging and extra care during anaesthesia is necessary
  20. 20. Connective Tissue Diseases
  21. 21. Systemic Lupus Erythematosus (1) SLE an “Systemic, inflammatory, multisystem disorder” Autoimmune disease in which auto-antibodies and immune complexes cause damage to organs. Epidemiology: High prevalence in Afro-carribeans, Peak age of onset 20- 40 years Aetiology: Hereditary , Genetic, Immune system disorder (Autoantibody production + impaired tolerance), UV light , Drugs.
  22. 22. Clinical features • Musculoskeletal 90% : -Symmetrical, small joint, arthralgia, - Jaccoud’s arthropathy –Joint deformity resembling RA – rare • Cutaneous 75% -Malar Rash: -Discoid Lupus • Neurology 60% -Cerebral Lupus • Lungs 50% -Recurent pleurisy and BILATERAL pleural effusions - “Shrinking Lung syndrome’ • Renal 30% (Histological changes are frequent) - Regular screening for urine for blood and protein required
  23. 23. ‘ A RASH POIN MD’ • Arthralgia • Renal problems • Anti-nuclear antibodies • Serositis • Haematological disorder • Photosensitivity • Oral ulcers • Immunological disorder • Neurological disorder • Malar rash • Discoid rash • >4 out of 11 needed
  24. 24. Investigations
  25. 25. Investigations • Bloods FBC: Leucopenia, lymphopenia and/or thrombocytopenia ESR: Raised CRP: Normal U+E: Urea and Creatinine raised in advanced renal disease • Urine Testing: Protein in urine and RBC Caste • Complement: Low C3 Low C4 Imaging • CT– infarcts or haemorrhage with evidence of cerebral atrophy • MRI – lesions in white matter
  26. 26. Serum Antinuclear Antibodies (ANA) Positive >95% Double stranded DNA Specific for SLE Positive 60% ENA: Anti-Ro and Anti-LA Detected RF Positive in 40% of patients Anticardiolipin antibodies Present in 35-45% of patients Serological Syphilis Test False Positive 1/3rd Immunoglobulins ↑ (IgG and IgA) Investigation-Serology
  27. 27. A 32 year old pregnant woman with a history of malar rash, photosensitivity and joint pain in her hands is seen in antenatal clinic. An appropriate autoantibody screen is performed. Which of following autoantibodies are most associated with poor fetal cardiac function with respect to this patient? • Anti-dsDNA • Antinuclear • Antiphospholipid • Anti-Ro • Anti-La
  28. 28. Management • Symptomatic treatment -NSAIDS -Antimalarials: e.g hydroxychloroquine • Corticosteroids and immunosuppressive drugs -Single dose I.M corticosteroids for flares -immunosuppressive drugs for severe thrombocytopenia, renal and cerebral diseases.
  29. 29. What is the best test to distinguish between a lupus flare and an infection in a patient with lupus?
  30. 30. Answer ESR and CRP ESR increased, CRP normal in flare ESR and CRP both increased in infection
  31. 31. Drugs that worsen SLE • Penicillamine – what is this? • Procainamide – what is this? • Hydralazine – what is this? • All associated with drug induced lupus • Which antibody is associated with drug induced lupus?
  32. 32. Sjogren’s Syndrome 2 types: Primary Sjogren’s: 9:1 F:M ratio Secondary Sjogren’s: Associated with CT diseases: RA, SLE, Systemic Sclerosis Classic Features 1.Decreased tear production —Dry eyes —Keratoconjunctivitis sicca 2.Decreased salivation —Dry mouth —Caries Other features: —Arthritis, Raynaud’s , Interstitial Nephritis
  33. 33. Investigations • Measure the affected areas: Conjunctival dryness • “Schirmer’s Test”: Filter paper under lower lid, measure distance along paper that tears are absorbed 5mm in 5mins = +ve • Blood tests:FBC: Increased Ig Autoantibodies: Anti-Ro + Anti-La(70%), RF+, ANA+(70%) Treatment •No cure •Symptomatic relief: Eye drops (artificial tears) Gels, frequent drinks, sugar free gum/pastilles etc. NSAIDs and immunosuppressants in severe systemic disease
  34. 34. A 42 year old woman presents to her GP with dry eyes, dry mouth and joint pain. She undergoes a Schirmer’s test which reveals a tear absorption rate of 3mm in 5 minutes. Concerning the diagnosis, which of following is most true? • Patients are at an increased risk of non-Hodgkin’s lymphoma • Women are three times more likely to be affected than men • The peak onset of age is in the 6th decade of life • Rheumatoid factor is positive in 70% of patients • Is associated with anti-mitochondrial antibodies
  35. 35. Systemic Sclerosis
  36. 36. What is it? • Chronic multisystem disease • Mainly affects skin and associated with Raynaud’s • F:M 3-5:1 • Presents between age 30-50 • 2 main types: i. Limited cutaneous scleroderma ii. Diffuse cutaneous scleroderma
  37. 37. Clinical Features 1. Limited cutaneous scleroderma (60%) – Initially get Raynaud’s – Followed by slow skin changes - hands, feet and forearms – Thickened skin – (Formerly known as CREST syndrome) – Calcinosis, Raynauds, Esophagitis, Sclerodactly, Telangiectasia
  38. 38. Clinical Features 2. Diffuse cutaneous scleroderma (40%) – Initially get Raynaud’s – More rapid and widespread skin changes – Also affects: 1. Oesophagus (like limited type) 2. Lungs: Pulmonary fibrosis , pulmonary vascular disease, causing pulmonary HTN 3. Kidneys: Acute or chronic renal failure 4. Heart: pericarditis, pericardial effusion, myocarditis, arrythmias
  39. 39. Investigations • Blood tests: – FBC: normochromic, normocytic anaemia, raised ESR – Autoantibodies (NOT present in all): • Limited cutaneous: anti-centromere • Diffuse cutaneous: anti-Scl70, anti-RNA pol I + III • Radiology: XR, CT and barium swallow • Oesophageal manometry
  40. 40. Treatment • No cure • Symptomatic relief: – Immunosuppression with iv cyclophosphamide – ACE inhibitors or ATII receptor antagonists for HTN – Hand warmers/ Ca channel blockers for Raynaud’s
  41. 41. Overview Polymyositis is a rare, subacute inflammatory myopathy.
  42. 42. OverviewOverview • Very rare (2-10/million); ♀ > ♂ • Subacute: weeks to months with typical delay before presentation • Inflammation of striated muscle • Aetiology complex and uncertain
  43. 43. Clinical featuresClinical features • Proximal muscle weakness: – Progressive – Symmetrical • Which sort of movements are initially affected? • Distal weakness may occur later • Ocular and other facial muscles spared even in advanced, untreated cases
  44. 44. Clinical Features • Respiratory, oesophageal & laryngeal muscles can be affected • Weakness is associated with wasting. • Isolated Polymyositis is incredibly rare. More commonly it is associated with either: – Systemic autoimmune or connective tissue disease – Viral or bacterial infection • Systemic symptoms including Raynaud’s phenomenon.
  45. 45. InvestigationsInvestigations 1 CK – raised (up to 50-fold) 2 Electromyography (EMG) show abnormalities. 3 Muscle biopsy. This is the definitive test
  46. 46. Management • Goal is to improve muscle strength. • First-line is 3 month trial of oral Prednisolone. This fails in 75% of patients • If this fails, other immunosuppressive drugs used. e.g. methotrexate, azathioprine, mycophenolate • If immunosuppression fails, rethink diagnosis. (Repeat biopsy!)
  47. 47. Any questions?
  48. 48. Take-home messages SLE is a systemic, inflammatory, multisystem disorder. It is an autoimmune disease in which auto-antibodies and immune complexes cause damage to organs. Sjogren’s Syndrome is a chronic inflammatory condition due to immunologically mediated destruction of epithelial exocrine glands Systemic Sclerosis is a chronic multisystem disease. It mainly affects skin and is associated with Raynaud’s Polymyositis is a subacute inflammatory myopathy of a proximal-to-distal progressive symmetrical pattern
  49. 49. Gout • What do you know?
  50. 50. Gout • Hyperuricaemia and uric acid crystals • Predilection for MTP joint (70%) • Presenting features? • Risk factors
  51. 51. Gout Risk Factors • Alcohol • Drugs – which ones? • High purine diet • High cell turnover • CKD
  52. 52. Ix • MUST RULE OUT SEPTIC ARTHRITIS • Joint aspirate – negatively birefringent needle shaped crystals under polarised light • X-ray • U+Es • Serum uric acid – may be normal in acute attack
  53. 53. A 67 year old man with gastro-oesophageal reflux disease (GORD) presents with an acutely painful swollen right metatarsophalangeal joint. Joint aspiration reveals the presence of needle shaped crystals that are negatively birefringent when viewed under polarised light. Which of the following is the most appropriate initial treatment option in this patient? • Colchicine • Indomethacin • Allopurinol • Steroids • Febuxostat
  54. 54. Rx • NSAIDs /colchicine/ steroids for acute phase • Allopurinol/ Febuxostat/ Rasbicurase for chronic phase • What if patient is on azathioprine?
  55. 55. Azathioprine toxicity • Nausea • Fatigue • Rash • Hair loss • BONE MARROW SUPPRESSION • Need to measure thiopurine S-methyltransferase levels before you start a patient on AZT
  56. 56. Pseudogout • What do you know?
  57. 57. Pseudogout • Calcium pyrophosphate crystals • Tends to affect larger joints • Risk factors?
  58. 58. Pseudogout risk factors • Hypomagnesaemia • Hypophosphataemia • HAEMOCHROMATOSIS • WILSON’S DISEASE • HYPERPARATHYROIDISM
  59. 59. Ix • MUST RULE OUT SEPTIC ARTHRITIS • Polarised light microscopy of joint fluid • X-ray • Consider secondary causes (esp if under 60) • Calcium • Magnesium • Iron studies • Caeruloplasmin
  60. 60. Practice SBA’s 1. Which one of the following systems is most commonly affected in SLE? A. Joints B. Skin C. Chest D. Kidney E. Heart
  61. 61. Clinical features • Musculoskeletal 90% : -Symmetrical, small joint, arthralgia, - Jaccoud’s arthropathy –Joint deformity resembling RA – rare • Cutaneous 75% -Malar Rash: -Discoid Lupus • Neurology 60% -Cerebral Lupus • Lungs 50% -Recurent pleurisy and BILATERAL pleural effusions - “Shrinking Lung syndrome’ • Renal 30% (Histological changes are frequent) - Regular screening for urine for blood and protein required
  62. 62. 2.You review the hands of a 60-year-old man who is complaining of 'arthritis' in his hands:
  63. 63. A. Rheumatoid arthritis B. Systemic Sclerosis C. Systemic Fungal infection D. Psoriatic Arthritis E. Reiter's Syndrome
  64. 64. 3. A 49 year old man suddenly experiences severe pain in his left ankle while walking. His past medical history is unremarkable, except for a recent course of antibiotics for gastroenteritis. Clinical examination reveals an inability to plantar flex the left foot. Which of the following antibiotics was the patient most likely to have been taking? • Erythromycin • Metronidazole • Amoxicillin • Cefalexin • Ciprofloxacin
  65. 65. 4. A 57-year-old man presents to his GP due to pain in his right knee. An x-ray shows osteoarthritis. He has no past medical history of note. What is the most suitable treatment option for the management of his pain? A.Oral Doclofenac with omeprazole B.Oral glucosamine C.Oral Diclofenac D.Oral Ibuprofen E.Oral paracetamol
  66. 66. 5. You refer a 24-year-old female to rheumatology with intermittent pain and swelling of the metacarpal phalangeal joints for the past 3 months. An x-ray shows loss of joint space and soft-tissue swelling. Rheumatoid factor is positive and a diagnosis of rheumatoid arthritis is made. What initial management is she most likely to be given to help slow disease progression? A.Infliximab B.Methotrexate C.Sulfasalazine D.Methotrexate + sulfasalazine + short-course of prednisolone E.Diclofenac
  67. 67. Rheumatoid Arthritis (6) Management 2009 NICE guidelines it is recommend that patients with newly diagnosed active RA start a combination of DMARDs (including methotrexate and at least one other DMARD, plus short-term glucocorticoids) 1.DMARDS Methotrexate (s.e derange LFT’s renal, neutopenia, PF, teratogenic) •Sulfasalazine (s.e infertility, G6PD heamolysis) •Lefunimide Others e.g Azathriopine, Gold (Also add prednisolone)
  68. 68. 6. A 25-year-old man presents complaining of dysuria and pain in his left knee. Three weeks previously he had suffered a severe bout of diarrhoea. What is the most likely diagnosis? A.Reactive Arthritis B.Disseminated gonococcal infection C.Bechet’s disease D.Ulcerative colitis E.Rheumatoid arthritis
  69. 69. 7. A 38 year old intravenous drug user presents with an acutely painful swollen left knee and a temperature of 38.30 C. Joint aspiration reveals a turbid, yellow fluid which is sent for culture. Which of the following is the most appropriate initial empirical treatment? • Intravenous flucloxacillin and intravenous benzylpenicillin • Oral metronidazole • Oral amoxicillin and oral erythromycin • Intravenous cefotaxime and intravenous gentamycin • Oral flucloxacillin and oral penicillin V
  70. 70. 8 A 72 year old lady presents with a two year history of right hip pain. She mentions that it is particularly troublesome after she has walked to the shops. X-ray shows loss of joint space and subchondral sclerosis and cysts. Which of the following is the most effective treatment for this lady? • Physiotherapy • Weight loss • Joint replacement • NSAIDs • Glucosamine and chondroitin sulphate
  71. 71. 9. Which one of the following is least associated with systemic lupus erythematous? A.Anti-nulcear antibodies B.Anti-Sm Antibodies C.Elevated ESR D.Elevated C3 And C4 levels E.Elevated anti-dsDNA titres in active disease
  72. 72. SLE: ANA is 99% sensitive - anti-Sm & anti-dsDNA are 99% specific C3, C4 are low in SLE not elevated
  73. 73. 10. You are doing the annual review of a 50-year-old woman who has rheumatoid arthritis. Which one of the following complications is most likely to occur as a result of her disease? A. Chronic lymphocytic leukaemia B. Hypertension C. Colorectal Caner D. Type 2 diabetes mellitus E. Ischaemic heart disease
  74. 74. 11 A 38 year old woman with known rheumatoid arthritis presents with a history of shortness of breath, easy bruising and recurrent chest infections. On clinical examination there is splenomegaly. Which of the following is the most likely explanation for this presentation? • Sulphasalazine • Methotrexate • Penicillamine • Felty’s syndrome • All of the above
  75. 75. 12 A 50 year old woman undergoes a DEXA bone scan which reveals a T score of -2.6. Taking the history reveals a strong family history of breast cancer and a 30 pack year smoking history. Which of the following treatment options is most appropriate for this patient? • Alendronate • Strontium Ranelate • Vitamin D • Calcitonin • Raloxifene
  76. 76. Can you any name anti-osteoporotic medications and their side effects?
  77. 77. • Anti-SSA • Anti-topoisomerase-I • Anti-centromere • Anti-liver kidney microsomal • Anti-Jo1