多发性肌炎和皮肌炎课件

Polymyositis and Dermatomyositis General situation Etiology and Pathogenesis Clinical Presentation Diagnosis and differential Diagnosis Treatment and prognosis General situation Classification of the Idiopathic inflammatory myopathies： Polymyositis,PM Dermatomyositis,DM Juvenile dermatomyositis DM、PM associated with malignancy Another autoimmine rheumatic disease Inclusion body Epidemiology Incidence:0.5/10万8/10万 Onset age：5 14 and 45 64 Sex：female:male=2:1 Race:Black:White=4:1 Pathogenesis Evidences of auto-antibodies MSAs(myositis-specific auto-antibodies) Antisynthetase：Anti-Jo 1 Anti-SRP(signal recognition particle) Anti-Mi2： Evidences of autoimmune reaction Often overlap other autoimmune diseases Immunized animal Response to corticosteroides. Etiology Susceptible Genes HLA-DR3 PM and Juvenile DM HLA-DR52 Jo-1,Anti-SRP HLA-DR7 Anti-Mi-2 Infection Onset season: Antisynthetase syndrome,Anti-SRP Same sequence of amino acid： Related virus: Influenza, hepatitis A and B, Coxsackievirus A9，B1 Autoimmune reaction PM：CD8+T MHC-I Cytokines：IL-1,IL2,IFN-r,TNF-a (TCR) V Va1,Va5,V1, V5 DM: infiltration of B cell. Environment Pathology Infiltrate of lymphocytic cells：T cell、 macrophages、plasma cell Necrosis of muscle fibers Muscle shrink Clinical Presentation Polymyositis,PM Symmetric proximal muscle Difficulty to get up from a sitting position Difficulty to reach over head Neck muscle： Upper esophageal： Respiratory tract: Myalgia Nonmuscular manifestations Pulmonary：Weakness of respiratory muscle, Aspiration Interstitial lung disease Pulmonary vasculitis Cardiac：Heart block, Arrythmias, Cardiomyopath Gastrointestinal Esophageal Stomach Small or large bowl dysmotility Arthritis: Dermatomyositis, DM Classic skin manifestations Erythematous around eye Gottonlesions “V” Erythematous on upper chest and neck Erythematous on shoulder hand of skilled worker Juvenile dermatomyositis(or polymyositis) Skin eruption Myositis Gastrointestinal vasculitis Lipodystrophy Ectopic Calcification Amyopathic dermatomyositis Gottron plus other characteristic skin eruption Biopsy Without the manifestations of myositis Excluding other inflammatory myositis (infection, drug) Laboratory Testing Elevated ESR, inosinic acid in blood and 24 urine Muscle enzymes： CPK , LDH CPK not elevated in PM or DM The early or later stage older patients Circulate inhibited CK Effusion of muscular cytomembrane Electromyography Muscle biopsy Auto-antibodies anti-synthetase syndrome: Jo-1 antibodies Interstitial lung disease Arthritis Raynauds phenomenon Hand of skilled worker Anti-SRP Anti-Mi-2 Diagnosis Symmetric proximal muscle Elevated muscle enzymes EMG Muscle biopsy Characteristic skin eruption Differential Diagnosis Inclusion body myositis Muscular dystrophies Neurologic conditions Guillain-Barre syndrome Myasthenia gravis：AChR Infectious myositis Drug-induced disorders Endocrine disorders Accompanied by another rheumatic disease or malignant Treatment Prednisone (12mg/kg.d) Immunosuppressive agents MTX, azathioprine, CTX IVIg(1-2g/kg