肌炎的臨床診斷肌炎的臨床診斷1臨床分型多發(fā)性肌炎嗜酸性粒細胞肌炎皮肌炎肉芽腫性肌炎免疫介導壞死性局灶/結(jié)節(jié)性肌炎肌炎眶周肌炎腫瘤相關(guān)性肌炎包涵體肌炎cTD相關(guān)性肌炎臨床分型2多發(fā)性肌炎(PM)ELEVATEDCKNomusclesymptomsDiagnosticWorkupofAsymptomaticHyperCKemiaNormalmuscleexamavoidexerciseSTOPCKlevel>1.5-2xULNforBlackmale>1200Blackfemale>65STOP/Non-blackmale>500observeNon-blackfemale>325心mmDiscusswithpatientRuleoutEMGandeutilityoffuturemusclebiopsyneuromuscularworkupcauses25-30%yieldSTOPandimitedtreatmentNormalEMG/BiopsytreatcausesdiopathicHypercKemia80%多發(fā)性肌炎(PM)3皮肌炎(DM)典型皮疹有診斷特異性合并D常見且進展快難治性皮疹考慮合并腫瘤可能皮肌炎(DM)4無肌病性皮肌炎(ADMClinicallyAmyopathicDM(CADMCADMClassicskinchanges>6mowithbiopsyNormalmuscleenzymesNoweaknessHypomyopathicAmyopathicEMGMuscleBiopsyMRIradiologicatleastoneabnormal)無肌病性皮肌炎(ADM5壞死性肌炎(NM)●是一組高度異質(zhì)性疾病Nearlyallpatientswillpresentwithsubacuteonsetofproximalweaknessthatisverysymmetricalandinvolvesthepelvicaswellastheshouldergirdle病理:肌肉的壞死是其突出臨床表現(xiàn),炎性浸潤可以很輕或不明顯預(yù)后因病因不同而不同壞死性肌炎(NM)6壞死性肌炎(NM)自身免疫性壞死性肌病①抗SRP相關(guān)②抗HMGCR相關(guān)③無自身抗體存在藥物相關(guān)壞死性肌病腫瘤相關(guān)壞死壞死性肌炎(NM)7壞死性肌炎(NM)MalignancyNecrotizingantibodyautoimmune(NAM)StatinOccasionalendominfillatOverlapHIVdiso,ses壞死性肌炎(NM)8壞死性肌炎(NM)AntiSRPantbodrelatedNAMatin-inducedimmuneNAParaneoplasticNAMAgeofonset399(2-72)N=24R圖316.3132635(4489)N=56708(38-87)N=13Re;[6.1112212a°,29,34Dysphagia[22]cialweakness21,29piratoryinvoivement[281Cardiaccomplications[1MyopathynotawaysparalelCreatinekinase:16167UL(3064-28008280U/L958-248562|U/L1700-24640N=15Re:332N=13Re(6121262934AlkainephosphataAlkalinephosphasestaininginpermysialconnectivetissue[3Isetoimmune.Variabletomsterodalone3,16Sterod+methotrexateResechon,steroids.[6,12.28torefractoryrequiringntunimabG,(29chemotherapyandhuxmab34allbeenusedMGrequredtnresponseinsamecases2a°l壞死性肌炎(NM)9壞死性肌炎(NMmyopathyAcuteonSlowonseNAMToxicmuscularagentsdystrophyAuto-AborAIDassociation?MuscularproteMSAOtherAuto-AbSRPAHMGCRAbSAABImmuno-fluorescencePm-SclAbStreatment][ParaneoplasicNM?][Auto:Ab?,AID[Dystrophy]treatmentstestandtre壞死性肌炎(NM10肌炎的臨床診斷課件整理11肌炎的臨床診斷課件整理12肌炎的臨床診斷課件整理13肌炎的臨床診斷課件整理14肌炎的臨床診斷課件整理15肌炎的臨床診斷課件整理16肌炎的臨床診斷課件整理17肌炎的臨床診斷課件整理18肌炎的臨床診斷課件整理19肌炎的臨床診斷課件整理20肌炎的臨床診斷課件整理21肌炎的臨床診斷課件整理22肌炎的臨床診斷課件整理23肌炎的臨床診斷課件整理24肌炎的臨床診斷課件整理25肌炎的臨床診斷課件整理26肌炎的臨床診斷課件整理27肌炎的臨床診斷課件整理28肌炎的臨床診斷課件整理29肌炎的臨床診斷課件整理30肌炎的臨床診斷課件整理31肌炎的臨床診斷課件整理32肌炎的臨床診斷課件整理33肌炎的臨床診斷課件整理34肌炎的臨床診斷課件整理35肌炎的臨床診斷課件整理36肌炎的臨床診斷課件整理37肌炎的臨床診斷課件整理38肌炎的臨床診斷課件整理39肌炎的臨床診斷課件整理40肌炎的臨床診斷課件整理41肌炎的臨床診斷課件整理42肌炎的臨床診斷課件整理43肌炎的臨床診斷課件整理44肌炎的臨床診斷課件整理45肌炎的臨床診斷課件整理46肌炎的臨床診斷課件整理47肌炎的臨床診斷課件整理48肌炎的臨床診斷課件整理49肌炎的臨床診斷課件整理50肌炎的臨床診斷課件整理51肌炎的臨床診斷課件整理52肌炎的臨床診斷課件整理53肌炎的臨床診斷課件整理54肌炎的臨床診斷課件整理55肌炎的臨床診斷課件整理56肌炎的臨床診斷課件整理57肌炎的臨床診斷課件整理58肌炎的臨床診斷課件整理59肌炎的臨床診斷課件整理60肌炎的臨床診斷課件整理61肌炎的臨床診斷課件整理62肌炎的臨床診斷課件整理63肌炎的臨床診斷課件整理64肌炎的臨床診斷課件整理65肌炎的臨床診斷課件整理66肌炎的臨床診斷課件整理67肌炎的臨床診斷課件整理68肌炎的臨床診斷課件整理69肌炎的臨床診斷課件整理70肌炎的臨床診斷課件整理71肌炎的臨床診斷課件整理72肌炎的臨床診斷課件整理73肌炎的臨床診斷課件整理74肌炎的臨床診斷課件整理75肌炎的臨床診斷課件整理76肌炎的臨床診斷課件整理77肌炎的臨床診斷課件整理78肌炎的臨床診斷課件整理79肌炎的臨床診斷課件整理80肌炎的臨床診斷課件整理81肌炎的臨床診斷課件整理82肌炎的臨床診斷課件整理83肌炎的臨床診斷課件整理84肌炎的臨床診斷課件整理85肌炎的臨床診斷課件整理86肌炎的臨床診斷課件整理87肌炎的臨床診斷課件整理88肌炎的臨床診斷課件整理89肌炎的臨床診斷課件整理90肌炎的臨床診斷課件整理91肌炎的臨床診斷課件整理92肌炎的臨床診斷課件整理93肌炎的臨床診斷課件整理94肌炎的臨床診斷課件整理95肌炎的臨床診斷課件整理96肌炎的臨床診斷課件整理97肌炎的臨床診斷課件整理98肌炎的臨床診斷課件整理99肌炎的臨床診斷課件整理100肌炎的臨床診斷課件整理101肌炎的臨床診斷課件整理102肌炎的臨床診斷課件整理103肌炎的臨床診斷課件整理104肌炎的臨床診斷課件整理105肌炎的臨床診斷課件整理106肌炎的臨床診斷課件整理107肌炎的臨床診斷課件整理108肌炎的臨床診斷課件整理109肌炎的臨床診斷課件整理110肌炎的臨床診斷課件整理111肌炎的臨床診斷課件整理112肌炎的臨床診斷課件整理113肌炎的臨床診斷課件整理114肌炎的臨床診斷課件整理115肌炎的臨床診斷課件整理116肌炎的臨床診斷課件整理117肌炎的臨床診斷課件整理118肌炎的臨床診斷課件整理119肌炎的臨床診斷課件整理120肌炎的臨床診斷課件整理121肌炎的臨床診斷課件整理122肌炎的臨床診斷課件整理123肌炎的臨床診斷課件整理124肌炎的臨床診斷課件整理125肌炎的臨床診斷課件整理126肌炎的臨床診斷課件整理127肌炎的臨床診斷課件整理128肌炎的臨床診斷課件整理129肌炎的臨床診斷課件整理130肌炎的臨床診斷課件整理131肌炎的臨床診斷課件整理132肌炎的臨床診斷課件整理133肌炎的臨床診斷課件整理134肌炎的臨床診斷課件整理135肌炎的臨床診斷課件整理136肌炎的臨床診斷課件整理137肌炎的臨床診斷課件整理138肌炎的臨床診斷課件整理139肌炎的臨床診斷課件整理140肌炎的臨床診斷課件整理141肌炎的臨床診斷課件整理142肌炎的臨床診斷課件整理143肌炎的臨床診斷課件整理144肌炎的臨床診斷課件整理145肌炎的臨床診斷課件整理146肌炎的臨床診斷課件整理147肌炎的臨床診斷課件整理148肌炎的臨床診斷課件整理149肌炎的臨床診斷肌炎的臨床診斷150臨床分型多發(fā)性肌炎嗜酸性粒細胞肌炎皮肌炎肉芽腫性肌炎免疫介導壞死性局灶/結(jié)節(jié)性肌炎肌炎眶周肌炎腫瘤相關(guān)性肌炎包涵體肌炎cTD相關(guān)性肌炎臨床分型151多發(fā)性肌炎(PM)ELEVATEDCKNomusclesymptomsDiagnosticWorkupofAsymptomaticHyperCKemiaNormalmuscleexamavoidexerciseSTOPCKlevel>1.5-2xULNforBlackmale>1200Blackfemale>65STOP/Non-blackmale>500observeNon-blackfemale>325心mmDiscusswithpatientRuleoutEMGandeutilityoffuturemusclebiopsyneuromuscularworkupcauses25-30%yieldSTOPandimitedtreatmentNormalEMG/BiopsytreatcausesdiopathicHypercKemia80%多發(fā)性肌炎(PM)152皮肌炎(DM)典型皮疹有診斷特異性合并D常見且進展快難治性皮疹考慮合并腫瘤可能皮肌炎(DM)153無肌病性皮肌炎(ADMClinicallyAmyopathicDM(CADMCADMClassicskinchanges>6mowithbiopsyNormalmuscleenzymesNoweaknessHypomyopathicAmyopathicEMGMuscleBiopsyMRIradiologicatleastoneabnormal)無肌病性皮肌炎(ADM154壞死性肌炎(NM)●是一組高度異質(zhì)性疾病Nearlyallpatientswillpresentwithsubacuteonsetofproximalweaknessthatisverysymmetricalandinvolvesthepelvicaswellastheshouldergirdle病理:肌肉的壞死是其突出臨床表現(xiàn),炎性浸潤可以很輕或不明顯預(yù)后因病因不同而不同壞死性肌炎(NM)155壞死性肌炎(NM)自身免疫性壞死性肌?、倏筍RP相關(guān)②抗HMGCR相關(guān)③無自身抗體存在藥物相關(guān)壞死性肌病腫瘤相關(guān)壞死壞死性肌炎(NM)156壞死性肌炎(NM)MalignancyNecrotizingantibodyautoimmune(NAM)StatinOccasionalendominfillatOverlapHIVdiso,ses壞死性肌炎(NM)157壞死性肌炎(NM)AntiSRPantbodrelatedNAMatin-inducedimmuneNAParaneoplasticNAMAgeofonset399(2-72)N=24R圖316.3132635(4489)N=56708(38-87)N=13Re;[6.1112212a°,29,34Dysphagia[22]cialweakness21,29piratoryinvoivement[281Cardiaccomplications[1MyopathynotawaysparalelCreatinekinase:16167UL(3064-28008280U/L958-248562|U/L1700-24640N=15Re:332N=13Re(6121262934AlkainephosphataAlkalinephosphasestaininginpermysialconnectivetissue[3Isetoimmune.Variabletomsterodalone3,16Sterod+methotrexateResechon,steroids.[6,12.28torefractoryrequiringntunimabG,(29chemotherapyandhuxmab34allbeenusedMGrequredtnresponseinsamecases2a°l壞死性肌炎(NM)158壞死性肌炎(NMmyopathyAcuteonSlowonseNAMToxicmuscularagentsdystrophyAuto-AborAIDassociation?MuscularproteMSAOtherAuto-AbSRPAHMGCRAbSAABImmuno-fluorescencePm-SclAbStreatment][ParaneoplasicNM?][Auto:Ab?,AID[Dystrophy]treatmentstestandtre壞死性肌炎(NM159肌炎的臨床診斷課件整理160肌炎的臨床診斷課件整理161肌炎的臨床診斷課件整理162肌炎的臨床診斷課件整理163肌炎的臨床診斷課件整理164肌炎的臨床診斷課件整理165肌炎的臨床診斷課件整理166肌炎的臨床診斷課件整理167肌炎的臨床診斷課件整理168肌炎的臨床診斷課件整理169肌炎的臨床診斷課件整理170肌炎的臨床診斷課件整理171肌炎的臨床診斷課件整理172肌炎的臨床診斷課件整理173肌炎的臨床診斷課件整理174肌炎的臨床診斷課件整理175肌炎的臨床診斷課件整理176肌炎的臨床診斷課件整理177肌炎的臨床診斷課件整理178肌炎的臨床診斷課件整理179肌炎的臨床診斷課件整理180肌炎的臨床診斷課件整理181肌炎的臨床診斷課件整理182肌炎的臨床診斷課件整理183肌炎的臨床診斷課件整理184肌炎的臨床診斷課件整理185肌炎的臨床診斷課件整理186肌炎的臨床診斷課件整理187肌炎的臨床診斷課件整理188肌炎的臨床診斷課件整理189肌炎的臨床診斷課件整理190肌炎的臨床診斷課件整理191肌炎的臨床診斷課件整理192肌炎的臨床診斷課件整理193肌炎的臨床診斷課件整理194肌炎的臨床診斷課件整理195肌炎的臨床診斷課件整理196肌炎的臨床診斷課件整理197肌炎的臨床診斷課件整理198肌炎的臨床診斷課件整理199肌炎的臨床診斷課件整理200肌炎的臨床診斷課件整理201肌炎的臨床診斷課件整理202肌炎的臨床診斷課件整理203肌炎的臨床診斷課件整理204肌炎的臨床診斷課件整理205肌炎的臨床診斷課件整理206肌炎的臨床診斷課件整理207肌炎的臨床診斷課件整理208肌炎的臨床診斷課件整理209肌炎的臨床診斷課件整理210肌炎的臨床診斷課件整理211肌炎的臨床診斷課件整理212肌炎的臨床診斷課件整理213肌炎的臨床診斷課件整理214肌炎的臨床診斷課件整理215肌炎的臨床診斷課件整理216肌炎的臨床診斷課件整理217肌炎的臨床診斷課件整理218肌炎的臨床診斷課件整理219肌炎的臨床診斷課件整理220肌炎的臨床診斷課件整理221肌炎的臨床診斷課件整理222肌炎的臨床診斷課件整理223肌炎的臨床診斷課件整理224肌炎的臨床診斷課件整理225肌炎的臨床診斷課件整理226肌炎的臨床診斷課件整理227肌炎的臨床診斷課件整理228肌炎的臨床診斷課件整理229肌炎的臨床診斷課件整理230肌炎的臨床診斷課件整理231肌炎的臨床診斷課件整理232肌炎的臨床診斷課件整理233肌炎的臨床診斷課件整理234肌炎的臨床診斷課件整理235肌炎的臨床診斷課件整理236肌炎的臨床診斷課件整理237肌炎的臨床診斷課件整理238肌炎的臨床診斷課件整理239肌炎的臨床診斷課件整理240肌炎的臨床診斷課件整理241肌炎的臨床診斷課件整理242肌炎的臨床診斷課件整理2