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  • 1.  
  • 2.
    • 40 yr old mrs stella rani has come to the hospital with c/o difficulty in using all 4 limbs-last 7 months
    • Blackish discoloration face and hands-6 months
    • Difficulty in swallowing 2 months
    • Pain over the both great toes 1month
  • 3.
    • She was apparently normal 7 months ago when developed pain over the both thighs followed by fever, she took treatment, fever subsided in 3 days but she noticed weakness of both legs, mainly in the proximal region , which is insidious in onset and slowly progressive in nature
    • h/o difficulty in getting up from squatting and lying posture, climbing the stairs -7months
    • Difficulty in lifting the hand above the head, combing the hair and bringing the food to mouth -6 months
    • h/o difficulty in lifting the head from the pillow-4 months
    • Difficulty in mixing the food- 2 months
    • Difficulty in holding the chapels last 2months
  • 4.
    • h/o hyper pigmentation over the face, chest and neck-6 months, non itchy
    • h/o dysphagia .- last 2 months , started with solid food now even for liquid, progressive,
    • mainly during initiation of swallowing and associated with throat pain
    • h/o pain over the both great toes and 2 nd toes for last 1month
    • h/o excessive hair loss +
  • 5.
    • No h/o difficulty in turning in bed from side to side
    • No h/o diurnal variation of weakness
    • No h/o twitching of muscles
    • No h/o sensory symptoms, cerebellar and posterior column symptoms
    • No h/o seizure
  • 6.
    • No h/o ptosis, diplopia
    • h/o dryness of mouth +
    • No h/o dryness of eyes
    • h/o cough with expectoration, white colour, scanty sputum
    • h/o dypnea on exertion +
    • No h/o fever
    • No h/o Photosensitivity
    • No h/o Altered bowel and bladder habits
    • h/o Head ache+
    • No loss of appetite but loss of weight+
  • 7.
    • PAST HISTORY
    • She had PT 10 years back, completed ATT
    • No H/O similar complaints in the past
    • No H/O DM,SHT,BA,CAD
    • FAMILY HISTORY
    • married, has 4 children,
    • no h/o spontaneous abortion
    • no other family member suffering from similar illness
    • Menstrual history
    • RMP 3/30 DAYS
    • TREATMENT HISTORY
    • not on any chronic treatment
    • no h/o drug allergy
  • 8.
    • Pt moderately built and nourished
    • Conscious ,oriented ,afebrile
    • pallor+
    • No icterus/no cyanosis/no clubbing/no PE/no GLA
    • no thyromegaly
    • vitals -BP-150/90 mmHg, PR-86/min, RR-18/m regular, thoraco abdominal
  • 9.
    • Skin – dark red rusty erythematous lesions over the malar region, fore head, both shoulder, upper chest and back ( V sign, shawl sign present)
    • Erythematous lesion over the MCP and PIP- Gottron papules and sign +
    • Cold great toes with tenderness +
    • ORAL CAVITY- poor oral hygiene, ulcer over the left upper buccal mucosa , carie`s tooth
  • 10.  
  • 11.  
  • 12.  
  • 13.  
  • 14.
    • Higher function- normal
    • Cranial nerves- palatal movements equal on both side but sluggish, otherwise normal CN examination
    • Spino motor system
    • RT LT
    • bulk- generalized wasting+
    • Arm 28cm 28cm
    • Fore arm 24cm 24cm
    • Thigh 42cm 42cm
    • Leg 24cm 24cm
  • 15.
    • Tone - normal on both side
    • Power - shoulder 2/5 both side
    • Elbow 3/5 both side
    • wrist 4/5 both side
    • hand grip good
    • hip 2/5 ,knee 3/5, ankle 4/5
    • DTR preserved
    • Plantar b/l flexor , other superficial reflexes normal
    • Sensory system normal
    • No cerebellor sign
    • No involuntory movements
    • No fasiculation
    • Gait- waddling gait
  • 16.
    • CVS-
    • S1S2+, No murmur
    • RS-
    • NVBS+, No added sounds
    • ABDOMEN-
    • soft , Epigastric tenderness+
    • No organomegaly no FF
  • 17.
    • Inflammatory myopathy
    • Dermatomyositis
    • or
    • Dermatomyositis with overlap syndrome
    • Anemia
    • SHT
    • Old PT
  • 18.
    • T.Prednisalone 40 mg od
    • T.Ranitidine 150mg bd
    • Inj.Ampicillin 1g iv tid
    • Inj.Deriphylline 2cc iv bd
    • T.Amlodipine 5mg od
    • Supportive measures
  • 19. HB 9.4 RBS 122 TC 9200 UREA 18 DC P60L35E5 CREATININE 0.9 ESR 17/32 Na 136 MCV 86.7 K 3.9 MCH 25.5 Cl 98 MCHC 29.7 HCO3 23 PCV 24 PLATELETS 1.78LAcs URINE ROUTINE- ALB/sug-nil DEP-1-2 pc RBC 4.16 million
  • 20.  
  • 21.
    • Rheumatologist advised to transfer the patient to GGH for evaluation & management
  • 22.  
  • 23.  
  • 24.  
  • 25.  
  • 26.
    • CPK-35 RA factor-negative
    • ALT-316 CRP- negative
    • AST-114 ANA-POSITIVE
    • LDH-2461 anti dsDNA-NEGATIVE
    • CHL-262
    • TGL-287
  • 27.
    • ECG -WNL
    • ECHO -NORMAL STUDY
    • USG abdomen - normal study
    • X ray both hands-soft tissue swelling, no calcification, no osteoporotic lesion
    • X ray elbow-soft tissue swelling
    • HRCT chest- cystic ectasia of bronchi in the posterior segment of Rt upper lobe,
    • Ground glass opacity noted in the basal segments of both Lower Lobe L>R
  • 28.
    • DERMATOLOGY - confirm the DM
    • Neurology - inflammatory myopathy
    • EMG fixed on 13/4/10
    • Cardiac evaluation- normal
    • Chest physician opinion- DM with ILD, old PT seqeule
    • MGE - UGI scopy normal, colonoscopy fixed on 19/3/10, barium swallow to be done
    • Dental opinion -chronic gingivitis, traumatic ulcer over the left buccal mucosa
    • ENT opinion - no growth in the pharynx, vocal cords normal
  • 29.
    • Inflammatory myopathy
    • Dermatomyositis
    • ILD
    • ANEMIA
    • SHT
    • OLD PT SEQEULE
  • 30.
    • Steroids
    • Methotrexate/cyclophosphamide
    • Hydroxy chloroquine
    • She is improving…..
  • 31. Idiopathic InflammatoryMyopathies (IIM ) Heterogeneous group of autoimmune syndromes characterized by chronic muscle weakness and muscle inflammation, systemic complications
  • 32.
    • Adult polymyositis (PM)
    • Adult dermatomyositis (DM)
    • Juvenile myositis (DM >> PM)
    • Malignancy-associated myositis
    • Myositis in overlap with another rheumatic disease
    • Inclusion body myositis (IBM)
    • Other uncommon forms
  • 33.
    • Prevalence 1/100,000
    • Estimated annual incidence of 1.9-7.7 per million persons
    • Women more commonly affected 2:1
    • Peak age of onset 40-50
  • 34.
    • The cause is unknown, but it may result from either a viral infection or an autoimmune reaction
    • Some cases of DM are a paraneoplastic phenomenon , indicating the presence of cancer and is usually pre-existent, with removal of the cancer resulting in remission of the DM.
    • Genetic – HLA DR3,DR5,DR, a-TNF polymorphism
    • Immune – abnormal T cell activity
    • Infectious – viral agents, Toxoplasma, Borrelia
    • Drugs – Hydroxyurea, penicillamine, statin, quinidine, phenylbutazone
  • 35.
    • Dermatomyositis is probably caused by B cell and complement-mediated (terminal attack complex) vascular inflammation,
    • while polymyositis is caused by the direct cytotoxic effect of CD8 + lymphocytes on muscle.
    • However, other studies of cytokines suggest that some of the inflammatory processes may be similar.
    • A recent report has linked tumor necrosis factor (TNF) abnormalities with dermatomyositis.
  • 36.
    • Progressive, gradual (over many months), symmetric muscle weakness  difficulty with everyday tasks that require proximal muscles
    • Fine motor skills may be affected later in the disease course (but are common early in IBM)
    • Normal sensation
    • Pharyngeal and neck flexor muscles are often involved  may have dysphagia or head drop
    • May have respiratory involvement late in the presentation
    • Ocular and facial muscles are spared
    • Tendon reflexes preserved
    • Myalgia and muscle tenderness may occur, early
    • in the disease
  • 37.  
  • 38.
        • Heliotrope rash : purple discoloration on the upper eyelids with peri orbital edema
        • Gottron’s papules : raised scaly violaceous rash over PIP and MCP joints
        • Erythematous rash : occurs on knees, elbows, neck and anterior chest ( V sign ), back and shoulders ( shawl sign )
        • Raynaud’s phenomenon, subQ calcifications, periungal erythema
        • Mechanic`s hand - irregular, thickened, distorted lateral and palmar surface of fingers
  • 39.  
  • 40.  
  • 41.  
  • 42.  
  • 43.  
  • 44.  
  • 45.
      • CD8 + T cells invade muscle fascicles(endo mysial invation), surround healthy muscle fibers and result in phagocytosis and necrosis
  • 46.
      • B cell and CD4+ T cell infiltrates within the perivascular or interfascicular space, antibodies activate C3 and MAC complex  activation of cytokines and chemokines
  • 47.
    • Spinal muscular atrophy, ALS (use EMG to help)
    • Muscular dystrophies  develop over longer period
    • Guillain-Barre, polio, West Nile virus, neurotoxin  acute muscle weakness
    • AZT - causes a mitochondrial myopathy
    • Drug reaction- Amphotericin B, Heroin, Amiodarone, Colchicine, chronic laxative use, Glucocorticoids, Statins, Cyclosporine, Gemfibrozil, Alcohol, Cocaine
    • Steroid induced muscle weakness
    • Polymyalgia rheumatica , fibromyalgia  muscle tenderness, pain with movement
    • Connective tissue diseases
    • Endocrine –Hypo and hyperthyroidism
  • 48.
    • Muscle enzymes – CK, AST, ALT, LDH, aldolase
    • CK sometimes normal in active DM and IBM but always elevated in active PM
    • ESR and CRP are often normal and are not reliable indicator of disease activity
    • Antibodies
      • positive ANA in > 75%
      • Anti Jo-1: found in 25%, 80% with this positive have lung involvement (antihistidyl transfer RNA [t-RNA] synthetase)
      • Anti Mi-2: found in 5-10%, more common in dermatomyositis
      • Anti –SRP associated with rapidly progressive PM
    • EMG –
      • short duration, low amplitude polyphasic units on voluntary activation
      • increased spontaneous activity with fibrillations, complex repetitive discharges, positive sharp waves
    • Muscle biopsy
    • MRI can detect early and patchy involvement, can guide biopy
  • 49.
    • Symmetric proximal muscle weakness
    • Elevation of serum muscle enzymes: CK, aldolase, AST, ALT, LDH
    • Abnormal electromyogram (EMG)
    • Characteristic muscle pathology –
      • degeneration (breakdown) and regeneration (healing)
      • inflammatory cells attacking muscle
  • 50.
    • Muscle pain
    • Positive Anti-Jo-1
    • Arthritis/arthralgia
    • Systemic inflammatory signs: fever, CRP, ESR
    • 4+ of them = PM
    • Skin rash of DM: Gottron’s papules or sign, heliotrope rash
    • 4+ and skin rash = DM
  • 51.
    • Extramuscular manifestations –
      • Systemic : fever, weight loss, arthralgias and raynaud”s phenomenon
      • Joint contractures common in juvanile DM
      • GI : dysphagia (upper 1/3 striated muscle)
      • Cardiac : AV conduction defects, tachyarrhythmias, dilated cardiomyopathy, CHF
      • Pulmonary : respiratory muscle weakness, interstitial lung disease
      • calcinosis -Subcutanious calcifications common in JDM
  • 52.
    • Assoctation with Malignancy
      • Most common include ovarian, breast carcinoma, melanoma, colon, non-Hodgkin’s lymphoma
      • Nasopharyngeal cancer common in Asians
      • Search for an occult malignancy in all adult cases of DM should be done.
  • 53.
    • Can associated with systemic sclerosis or mixed connective tissue disease
    • Very rarely with RA , SLE or sjogren`s
    • DM with SS will have a specific anti nuclear antibody, anti-pm/scl, directed against a nucleolar protein complex
  • 54.
    • Antisynthetase syndrome
    • A subset of pts with PM and DM can have a group of findings including inflammatory arthritis, Raynaud`s phenomenon, ILD associated with certain auto antibodies( anti jo-1 )
    • Amyopthic DM or Dermatomyositis sine myositis
    • Skin lesion without muscle involvement
    • another subset of patients with dermatomyositis have controlled myopathy but continue to have severe and sometimes debilitating skin disease; this condition has been termed postmyopathic dermatomyositis .
  • 55.
    • Juvenile dermatomyositis is a systemic vasculopathy, affecting mainly the skin and muscle.
    • It differs from the adult form of dermatomyositis by the presence of vasculitis of the small blood vessels, which can involve the gastrointestinal tract and myocardium , besides skin and muscle.
    • Calcinosis is an additional feature that is present in juvenile dermatomyositis
    • Juvenile dermatomyositis is not associated with development of malignancies, unlike adult dermatomyositis.
  • 56.
    • Other features
    • Vasculitis and skin ulceration
      • Myocarditis with arrhythmias can occur
      • Arthralgia/arthritis with contractures
      • Muscular contractures  decreased joint mobility
      • Gastrointestinal dysfunction
      • Pulmonary involvement
      • Calcinosis (after 1—2 years )
  • 57.  
  • 58.
    • White, male, and over the age of 50
    • Onset more insidious than PM or DM –over years
    • Asymmetric distal motor weakness is common
    • CK- mostly normal
    • Muscle biopsy-intracellular vacuoles with tubular or filamentous inclusions in the nucleus or cytoplasm
  • 59.
    • Glucocorticoids: start with high dose prednisone 1 mg/kg/day, taper after 4 weeks until lowest dose possible reached
      • Caution as long term use may cause muscle weakness, osteoporosis
      • 75% will require additional medications
    • Immunosuppressants : Azathioprine-3mg/kg daily, Methotrexate-7.5mg/wk, Cyclophosphamide-0.5-1g/monthly, Chlorambucil, Cyclosporine, Mycophenolate mofetil-2.5mg/day
    • Immuno modulators – IV Ig –improved not only strength but also the underlying immuno pathology, benefit short lived, Repeated infusion q6-8 weeks
    • Dose 2g/kg divided over2-5 days per course
    • Plasmapheresis – no statistical difference between those given immunosuppressants and those who received plasmapheresis
    • For skin rashes - Limit sun exposure, hydroxychloroquine
  • 60.
    • For both DM and PM, 5 year survival is 95%, 10 year survival is 84%
    • Death is usually due to pulmonary, cardiac or systemic complications
    • Poor prognostic factors – older age at diagnosis, ethnicity, bulbar involvement, delayed treatment, cardiac and pulmonary involvement
    • No correlation with prognosis - CPK level, grade of disability and degree of muscle weakness at presentation
  • 61.
    • Choy and Isenberg. “ Treatment of dermatomyositis and polymyositis”. Rheumatology . 2002; 41: 7-13.
    • Dourmishev and Wollina. “ Dermatomyositis: immunopathologic study of skin lesions”. Acta Dermatoven APA. Vol 15, No 1. 2006.
    • Harrison’s Internal Medicine, 17 th edition .
    • Miller, M. “Clinical manifestations and diagnosis of adult dermatomyositis and polymyositis” UpToDate Online. Last updated February 15, 2008.
    • Miller, M. and Rudnicki, S. “Initial treatment of dermatomyositis and polymyositis in adults.” UpToDate Online. Last updated June 5, 2008.
    • www.neuropathologyweb.org . Northeastern Ohio Universities College of Medicine.
  • 62.