08.06 多发性肌炎和皮肌炎.ppt

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,V?1, V?5 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