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Dermatomyositis and polymyositis

Dermatomyositis and polymyositis. By: Brittni McClellan. Classifications. Polymyositis/dermatomyositis - description. Description: Inflammatory Myopathy (Myositis) Symmetric proximal muscle weakness and rash

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Dermatomyositis and polymyositis

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  1. Dermatomyositis and polymyositis By: Brittni McClellan

  2. Classifications

  3. Polymyositis/dermatomyositis - description • Description: • Inflammatory Myopathy (Myositis) • Symmetric proximal muscle weakness and rash • Number of Conditions in which muscle becomes damaged by non-suppurative lymphocytic inflammatory process. • Pathophysiology: • Autoimmune • Genetics: • HLA-DQ0301 • Etiology: Unknown

  4. Polymyositis/dermatomyositis - description Pathophysiology: • Focal Infiltration of T-Cells • Dermatomyositis: Perimysial • Polymyositis: Endomysial • Small number of macrophages • Muscle fiber degeneration and atrophy

  5. Polymyositis/dermatomyositis – H&P • Patient History: • Fever • Anorexia/Weight Loss • Fatigue • Dysphonia • Weakness • Difficulty Rising from Floor • Difficulty Climbing Stairs • Difficulty Swallowing/Regurgitation through Nose • Rash (Dermatomyositis) • Diagnosis: Rash + 3 Additional Criteria • Progressive, Symmetric Weakness of Proximal Muscles • Dermatitis: • Heliotrope Rash over Eyelids • Gottron Papules over Extensor Surfaces of Joints • Elevated Muscle Enzymes • EMG: Myopathy and Denervation • Biopsy: Inflammatory Myositis • Physical Exam: • Above Symptoms • Nailfold Telangiectasia • Straight Leg Raise <20s

  6. Polymyositis/dermatomyositis - Diagnosis Imaging: • MRI: • Inflamed Muscles – Signal Enhancement • Used to direct biopsy • Video Swallow Study • Identify Palatal or Proximal Esophageal Weakness • Pulmonary Function Tests • Evaluates Pulmonary Musculature and Interstitial Lung Disease Diagnosis: • Autoantibodies: • Normal Rheumatoid Factor • Normal Complement • Normal dsDNA • ANA: 10-50% • PM-1: 60% of Adults, Rare in Children • Jo-1: Associated with Interstitial Lung Disease • Enzymes: Elevated in 95% of Cases • Creatinine Kinase • Aspartate Aminotransferase (AST) • Aldolase • Lactate Dehydrogenase (LDH)

  7. Polymyositis/dermatomyositis – treatment • Medication +/- Radiotherapy • Prednisone • 2 mg/kg/d for 1 month • Taper over months to years • IV Gamma Globulin (Rash) • Plaquinel (Rash) • Methotrexate • Cyclosporine • Mycophenolate Mofetil

  8. Polymyositis/dermatomyositis – treatment • Associated Conditions • Interstitial lung disease • Malignancy: Strong Association with Dermatomyositis, Less with Polymyositis • Breast or Ovarian Cancer • Prostate Cancer • Lung Cancer • Other Connective Tissue Diseases

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