多发性肌炎和皮肌炎课件

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1、PolymyositisandDermatomyositisGeneralsituationEtiologyandPathogenesisClinicalPresentationDiagnosisanddifferentialDiagnosisTreatmentandprognosisGeneralsituationClassificationoftheIdiopathicinflammatorymyopathies：Polymyositis,PMDermatomyositis,DMJuveniledermatomyositisDM、PMassociatedwithmali

2、gnancyAnotherautoimminerheumaticdiseaseInclusionbodyEpidemiologyIncidence:0.5/10万~8/10万Onsetage：5~14and45~64Sex：female:male=2:1Race:Black:White=4:1PathogenesisEvidencesofauto-antibodiesMSAs(myositis-specificauto-antibodies)Antisynthetase：Anti-Jo1Anti-SRP(signalrecognitionparticle)Anti-Mi2：

3、EvidencesofautoimmunereactionOftenoverlapotherautoimmunediseasesImmunizedanimalResponsetocorticosteroides.EtiologySusceptibleGenesHLA-DR3PMandJuvenileDMHLA-DR52Jo-1,Anti-SRPHLA-DR7Anti-Mi-2InfectionOnsetseason:Antisynthetasesyndrome,Anti-SRPSamesequenceofaminoacid：Relatedvirus:Influenza,he

4、patitisAandB,CoxsackievirusA9，B1AutoimmunereactionPM：CD8+TMHC-ICytokines：IL-1,IL2,IFN-r,TNF-a(TCR)VVa1,Va5,V1,V5DM:infiltrationofBcell.EnvironmentPathologyInfiltrateoflymphocyticcells：Tcell、macrophages、plasmacellNecrosisofmusclefibersMuscleshrinkClinicalPresentationPolymyositis,PMSymmetr

5、icproximalmuscleDifficultytogetupfromasittingpositionDifficultytoreachoverheadNeckmuscle：Upperesophageal：Respiratorytract:MyalgiaNonmuscularmanifestationsPulmonary：Weaknessofrespiratorymuscle,AspirationInterstitiallungdiseasePulmonaryvasculitisCardiac：Heartblock,Arrythmias,CardiomyopathGas

6、trointestinalEsophagealStomachSmallorlargebowldysmotilityArthritis:Dermatomyositis,DMClassicskinmanifestationsErythematousaroundeyeGottonlesions“V”ErythematousonupperchestandneckErythematousonshoulderhandofskilledworkerJuveniledermatomyositis(orpolymyositis)SkineruptionMyositisGastrointest

7、inalvasculitisLipodystrophyEctopicCalcificationAmyopathicdermatomyositisGottronplusothercharacteristicskineruptionBiopsyWithoutthemanifestationsofmyositisExcludingotherinflammatorymyositis(infection,drug)LaboratoryTestingElevatedESR,inosinicacidinbloodand24uri