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Polymyositis Dermatomyositis

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It is a slide show about the Inflammatory Muscle Diseases with particular focus on Polymyositis and Dermatomyositis.

Published in: Health & Medicine
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Polymyositis Dermatomyositis

  1. 1. MYOSITIS Zia ul Haq Jawad Munir
  2. 2. Definition Sabacute or slowly progressive, symmetrical weakness primarily affecting the proximal limb and trunk muscles.
  3. 3. Classification of Myositis Polymyositis Dermatomyositis Poly/Dermato-myositis associated with Malignancy Poly/Dermato-myositis associated with Connective Tissue Disease Inclusion Body myositis Amyopathic Dermatomyositis
  4. 4. EPIDEMIOLOGY Annual Incidence: 2-10 case /million Peak age : 10-15 and 45-55 years old Female: Male ratio: 3:1 total Black:White : 4:1
  5. 5. CLINICAL MANIFESTATIONS Weakness of muscles -shoulder girdle - pelvic girdle - neck flexors Myalgia is minimal Constitutional symptoms-fatigue, fever, weight loss
  6. 6. CLINICAL MANIFESTATIONS Rheumatic: Arthralgia/Arthritis 20-70% Pulmonary: Interstitial Lung Disease 10% GIT: Esophageal Dysmotility 10-30% Cardiac: Conduction Blocks and Arrhythmia Vascular: Raynaud’s Phenomenon 20-40% Skin : Rash, Livedo Reticularis
  7. 7. Myositis-PROGNOSIS 5-YEAR SURVIVAL IS AROUND 85% IN PM, DM, PM/DMASSOC. CTD. MUCH LOWER IN PM/DMASSOC. MALIGNANCY.
  8. 8. Polymyositis A persistent inflammatory muscle disease that causes weakness of the skeletal muscles, which control movement. Medically, polymyositis is classified as a chronic inflammatory myopathy — one of only three such diseases.
  9. 9. Polymyositis Can occur at any age, Adults -30s, 40s or 50s. Blacks Women
  10. 10. Polymyositis signs and symptoms usually develop gradually, over weeks or months. Remissions - rare Remissions: periods during which symptoms spontaneously disappear
  11. 11. Signs and symptoms appear gradually, Progressive muscle weakness Difficulty swallowing (dysphagia) Difficulty speaking Mild joint or muscle tenderness Fatigue Shortness of breath
  12. 12. Signs and symptoms affects the muscles closest to the trunk, particularly hips, thighs, shoulders, upper arms and neck. weakness is symmetrical worsens over time. As muscle weakness progresses, difficult to climb stairs, rise from a seated position, lift objects or reach overhead.
  13. 13. Complications Dysphagia Which in turn may cause weight loss and malnutrition. Aspiration pneumonia Shortness of breath or respiratory failure. Calcinosis Calcium deposits in muscles, skin and connective tissues
  14. 14. Associated conditions Polymyositis is often associated with other conditions that may cause further complications of their own, or in combination with polymyositis symptoms. Associated conditions include: Raynaud's phenomenon. Other connective tissue diseases. Cardiovascular disease. Lung disease.
  15. 15. DIAGNOSTIC CRITERIA 1. PROXIMAL MOTOR WEAKNESS: symmetric, proximal muscles 2. HIGH SERUM MUSCLE ENZYMES: CPK, aldolase, myoglobin, AST, ALT, LDH
  16. 16. DIAGNOSTIC CRITERIA 3. EDT= electro-diagnostic tests NEUROPATHIC DISORDER PM/DM -Poly-phasic action potentials -long duration -large amplitude -Poly-phasic action potentials -short duration -low amplitude EMG abnormalnormalNCV
  17. 17. DIAGNOSTIC CRITERIA 4. MUSCLE BIOPSY: biopsy a clinically weak muscle, contralateral to an abnormal muscle ( by EDT), MRI directed. a. Perivascular and endomysial inflammation CD8+ T cells in PM, CD8+, CD4+ T and B cells in DM b. b. Muscle fiber necrosis and regeneration
  18. 18. LABORATORY TESTS HIGH MUSCLE ENZYMES:- CPK ELEVATED ESR , CRP:- 50% POSITIVE ANA:- 50-80% AUTOANTIBODIES:- anti- RNP (MCTD) anti-PM/Scl (OVERLAP)
  19. 19. Myositis-specific AUTOANTIBODIES ANTI Jo-1 part of ANTI SYNTHETASE Ab’s Antibodies to the antigen- Aminoacyl-tRNA synthetase, in 20-50% of PM>>DM ANTI SRP = anti signal recognition particle In 5% of PM ANTI Mi-2 in 10% of DM.
  20. 20. Treatment 1. STEROIDS 2. IMMUNOSUPPRESSIVE AGENTS: methotrexate, azathioprine, cytoxan, cellcept 3. IMMUNOMODULATORY AGENTS: IVIG, Plasmapheresis 4. REHABILITATION
  21. 21. Cytokines in Inflammation Pro-inflammatory Anti-inflammatory TNFa IL-1b sTNFR IL-10 IL-1Ra
  22. 22. Macrophage or Activated T Cell Soluble TNFa Target Cell TNFa Receptor Signal Induction Synthesis and Function of TNFa Transmembrane TNFa Receptor-Bound TNFa
  23. 23. Adapted with permission from Choy EHS, Panayi GS. N Engl J Med. 2001;344:907-916. Copyright © 2001 Massachusetts Medical Society. All rights reserved. Inhibition of Cytokines Inflammatory cytokine Normal interaction Neutralization of cytokines Receptor blockade Activation of anti-inflammatory pathways Cytokine receptor Soluble receptor Monoclonal antibody Monoclonal antibody Receptor antagonist Anti-inflammatory cytokine Suppression of inflammatory cytokines No signal No signal Inflammatory signal
  24. 24. Humira- The first fully human antiTNFa Chimeric Antibody 70% Human Humanized Antibody 95% Human Fully Human Antibody 100% Human Mouse Human Chimeric Humanized Fully Human 24
  25. 25. Treatment corticosteroid When muscle strength improves, usually in 4 to 6 weeks, the medication is slowly tapered off. Maintenance therapy with prednisone may be continued indefinitely. DMARDs - If unresponsive to corticosteroids methotrexate and azathioprine,
  26. 26. Treatment Intravenous gamma globulin IVIG is a purified blood product that contains healthy antibodies from thousands of blood donors. The healthy antibodies in IVIG can block the damaging antibodies that attack muscle in polymyositis. Repeat infusions q 6-8 weeks
  27. 27. Other immunosuppressive medicine Tacrolimus (Prograf) is a transplant-rejection drug that may work to inhibit the immune system. Immunosuppressants, cyclophosphamide (Cytoxan) and cyclosporine (Gengraf, Neoral, Sandimmune),
  28. 28. Biological therapies Rituximab (Rituxan) Tumor necrosis factor (TNF) inhibitors etanercept (Enbrel) and infliximab (Remicade),
  29. 29. Nursing management Coping and support Educate about the illness Balance Rest and exercise
  30. 30. Myositis associated with skin diseases
  31. 31. Dermatomyositis a muscle disease characterized by inflammation and a skin rash. It is a type of inflammatory myopathy. 5 - 15 and adults age 40 - 60. Women Polymyositis is a similar condition, but the symptoms occur without a skin rash. Maria Carmela L. Domocmat, RN, MSN
  32. 32. Symptoms Dysphagia Muscle weakness, stiffness, or soreness Purple or violet colored upper eyelids Purple-red skin (violaceous) rash SOB Maria Carmela L. Domocmat, RN, MSN
  33. 33. Symptoms The muscle weakness may appear suddenly or develop slowly over weeks or months. may have difficulty raising arms over head, rising from a sitting position, and climbing stairs. The rash may appear over the face, knuckles, neck, shoulders, upper chest, and back. Maria Carmela L. Domocmat, RN, MSN
  34. 34. reddish-purple (violaceous) rash reddish-purple (violaceous) rash. The rash is named after the tendency of plants to grow toward the sun (heliotropic) and is characteristic of dermatomyositis. Maria Carmela L. Domocmat, RN, MSN
  35. 35. purple (violaceous) plaques The appearance of purple (violaceous) plaques on the knees may be associated with dermatomyositis. Maria Carmela L. Domocmat, RN, MSN
  36. 36. Gottron's sign Red, thickened, scaly skin over the knuckles Maria Carmela L. Domocmat, RN, MSN
  37. 37. Heliotrope eyelids eyelids develop a brown (violaceous - rather than red) color. Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis. Maria Carmela L. Domocmat, RN, MSN
  38. 38. violet-colored inflammation (erythema) over the knuckles Maria Carmela L. Domocmat, RN, MSN
  39. 39. periungual erythema Candida paronychia produced periungual erythema, edema and nail fold maceration. Maria Carmela L. Domocmat, RN, MSN
  40. 40. Dx Exams CPK & aldolase ECG Electromyography Magnetic resonance imaging (MRI) Muscle biopsy Maria Carmela L. Domocmat, RN, MSN
  41. 41. Treatment Corticosteroids Immunosuppressants When muscle strength gets better – taper off corticos However, most people take prednisone indefinitely. If the condition is associated with a tumor, the muscle weakness and rash may improve when the tumor is removed. Maria Carmela L. Domocmat, RN, MSN
  42. 42. Outlook (Prognosis) Some recover and have symptoms completely disappear - especially in children. In adults, death may result from severe and prolonged muscle weakness, malnutrition, pneumonia, or lung failure. The major causes of death are cancer (malignancy) and lung disease. Maria Carmela L. Domocmat, RN, MSN
  43. 43. Possible Complications Acute renal failure Cancer (malignancy) Inflammation of the heart Joint pain Lung disease Maria Carmela L. Domocmat, RN, MSN
  44. 44. DIFFERENTIAL DIAGNOSIS -MYOPATHY- DRUG/TOXIN -NEURO-MUSCULAR -ENDOCRINE DISEASE -INFECTIOUS MYOSITIS -METABOLIC STORAGE MYOPATHIES -MITOCHONDRIAL MYOPATHY -OTHERS
  45. 45. ANTI-SYNTHETASE SYNDROME associated with anti-Jo1 antibodies with acute onset of PM>> DM disease. Associated with ILD -40-60%, deforming and non-erosive arthritis, Mechanic’s hands and Raynaud’s phenomenon
  46. 46. Steroid response prognosisHLAClinical association prevalenceAb’s moderatemoderateDR3Anti- synthetase syndrome 20-50% (PM) Anti- Jo-1 poorbad (cardiac) DR5Severe PM5% (PM) Anti- SRP goodgoodDR7Classical DM 5-10% (DM) Anti- Mi-2 Myositis-specific AUTOANTIBODIES
  47. 47. Poly/Dermato-myositis associated with malignancy Associated neoplasms present within the first 2 years of PM/DM followup In PM- 10 % In DM- 15 % Reports of: carcinoma-lung, stomach, ovary lymphoma Routine screening in DM

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