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Inflammatory disease of muscle

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Inflammatory disease of muscle

  1. 1. 醫學系「免疫與感染學 V 」模組 Inflammatory Diseases of Muscle -- Polymyositis & Dermatomyositis 魏正宗 中山醫學大學附設醫院 過敏免疫風濕科主任、中藥臨床試驗中心主任 Tel: 04 24729595 ext 34314; Email: wei3228@gmail.com
  2. 2. 學習目標  Inflammatory Diseases of Muscle  Definition Pathophysiology  Clinical manifestations  Diagnosis  Treatment  Prognosis 
  3. 3. Weakness, etiologies: Nerve 2. Neuromuscular junction 3. Muscle 4. Psychogenic 1.
  4. 4. 5 subsets of inflammatory myopathies
  5. 5. Proximal muscle weakness  Shoulders, thighs, trunk and pelvic girdle muscles are most affected.  Never facial and ocular muscles  Esophagus, pharynx and respiratory muscles may involved.  May arthralgia and myalgia
  6. 6. Heliotrope sign
  7. 7. Heliotrope sign:
  8. 8. 皮肌炎 Dermatomyositis
  9. 9. V-sign: rash over anterior neck and upper chest
  10. 10. Gottron’s sign
  11. 11. Gottron’s sign
  12. 12. Gottron’s sign and periungual erythema
  13. 13. Periungual erythema
  14. 14. Vasodilatation in capillary microscope
  15. 15. Mechanist hand
  16. 16. Lab  Muscle enzyme–    Creatine Kinase Indicator of disease activity and severity May be normal in very early, remission or atrophy pts. CK rise weeks preceding clinical evidence of muscle weakness  Other muscle enzymes: ALT, AST, LDH, aldolase
  17. 17. Autoantibodies   ANA, anti-nuclear antibody ~80% Anti-ENA, extractable nuclear antigen  Anti-Jo-1 • most common myositis-specific autoantibody ~30%. • anti–tRNA synthetase syndrome: Lung iinvolvement, Raynaud's phenomenon  Anti-SRP antibodies • signal recognition particle (SRP) : translocation of newly synthesized proteins into the endoplasmic reticulum. • only in polymyositis with severe myopathy and aggressive disease  Anti-Mi-2 antibodies • • directed against a helicase involved in transcriptional activation associated with the relatively acute onset of classic dermatomyositis with erythroderma and the shawl sign
  18. 18. EMG of PM/DM • Triads in 40% of pts: polyphasic, small amplitude, short duration • Chracteristic but not diagnostic • 15% of pts may be normal EMG
  19. 19. Muscle biopsy of polymyositis •Focal and endomysial infiltration of T cell, esp. CD8, with small number of macrophage •Muscle fiber degeneration and atrophy
  20. 20. Muscle biopsy of dermatomyositis  Peri-vascular infiltration of inflammatory cells, composed of higher percentage of B cell and CD4 T cells.
  21. 21. Image study  MRI  Muscle scan
  22. 22. Other systemic features of idiopathic inflammatory myopathy  Esophageal     Gastrointestinal     Uncoordinated peristalsis Reflux Dysphagia Uncoordinated peristalsis Delayed gastric emptying Ischemic necrosis Endocrine   Autoimmune thyroiditis “euthyroid sick “syndrome
  23. 23. Cardiopulmonary features  Cardiovascular manifestations     Raynaud’s phenomenon Myocarditis Conduction abnormalities Heart failure  Pulmonary manifestation    Diffuse interstitial disease Pulmonary hypertension Bronchiolitis obliterans organizing pneumonia
  24. 24. Raynaud’s phenomenon
  25. 25. Diagnostic criteria of Dermatomyositis/Polymyositis 1. 2. 3. 4. 5. Proximal muscle weakness Increased muscle enzyme(CK) EMG findings Muscle biopsy Skin lesion -- Gottron's sign, heliotrope sign
  26. 26. Myositis in autoimmune diseases  Common in scleroderma, SLE, MCTD, Sjogren’s syndrome  Sometimes in RA, Adult Still’s disease, Vasculitis syndrome. Esp. Wegner’s granulomatosis and polyarteritis nodosa.
  27. 27. Myositis association with malignancy  Frequency of malignancy in myositis derived from 5%-35%  Most common sites: NPC, Lung, GI, Gyn  Work-up: careful history and PE and routine laboratory, tumor markers, image studies
  28. 28. Approaching DM/PM
  29. 29. Management of PM/DM  Corticosteroid alone (60-100 mg/day)  Immunosuppressive agents in conjunction with corticosteroid therapy Hydroxychloroquine (100-200 mg/day)  Azathioprine (2 mg/kg/d-50 mg/d)  Methotrexate (MTX 7.5-25 mg/wk)  Cyclphosphamide (50-200mg/d)  Cyclosporin A (7.5-10mg/kg/d) 
  30. 30. Steroid in Myositis  prednisolone1-2 mg/kg/day or methylprednisolone pulse therapy  90% improved after steroid therapy in weeks to months  50-75% had complete remission  Need slowly tapering of steroid  Tends to occur when taper steroid
  31. 31. Resistant disease - Rituximab (anti-CD20) - Intravenous immune globulin (IVIG) Mycophenolate mofetil Tumor necrosis factor inhibitors
  32. 32. Unfavorable Outcome         Malignancy Other collagen vascular disease Raynaud’s phenomenon Dysphagia Older age Severe or refractory muscle disease Cardiac involvement Pneumonitis/Interstitial lung disease
  33. 33. Conclusions  Workup of muscle weakness  Differential diagnosis of myopathy  Classification and diagnostic criteria of dermatomyositis and polymyositis  Has specific autoantibodies  Can be controlled by steroid and steroidsparing agents
  34. 34. References  eMedicine : Polymyositis/Dermatomyositis http://www.emedicine.com/neuro/topic85.htm  UpToDate : Polymyositis/Dermatomyositis http://www.lib.csmu.edu.tw/overlib/2305.php

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