肌炎的臨床診斷課件整理

肌炎的臨床診斷臨床分型多發(fā)性肌炎嗜酸性粒細(xì)胞肌炎皮肌炎肉芽腫性肌炎免疫介導(dǎo)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)性肌炎多發(fā)性肌炎(PM)ELEVATEDCKNomusclesymptomsDiagnosticWorkupofAsymptomaticHyperCKemiaNormalmuscleexamavoidexerciseSTOPCKlevel>1.5-2xULNforBlackmale>1200Blackfemale>65STOP/Non-blackmale>500observeNon-blackfemale>325心mmDiscusswithpatientRuleoutEMGandeutilityoffuturemusclebiopsyneuromuscularworkupcauses25-30%yieldSTOPandimitedtreatmentNormalEMG/BiopsytreatcausesdiopathicHypercKemia80%皮肌炎(DM)典型皮疹有診斷特異性合并D常見且進(jìn)展快難治性皮疹考慮合并腫瘤可能無肌病性皮肌炎(ADMClinicallyAmyopathicDM(CADMCADMClassicskinchanges>6mowithbiopsyNormalmuscleenzymesNoweaknessHypomyopathicAmyopathicEMGMuscleBiopsyMRIradiologicatleastoneabnormal)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)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)MalignancyNecrotizingantibodyautoimmune(NAM)StatinOccasionalendominfillatOverlapHIVdiso,ses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(NMmyopathyAcuteonSlowonseNAMToxicmuscularagentsdystrophyAuto-AborAIDassociation?MuscularproteMSAOtherAuto-AbSRPAHMGCRAbSAABImmuno-