皮肌炎特异抗体与特定的病理表现相关
作者：Pinal-FernandezI,翻译：张警丰
发布者：武东审校者：金银姬
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目的
皮肌炎（DM）相关自身抗体与临床表型相关。本研究的目的是评估这些自身抗体与特异性肌活检表现的相关性。
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方法
在约翰.医院住院的曾行肌活检的皮肌炎患者，检测其自身抗体如Mi-2,TIF1-γ,NXP2,MDA5,Ro52,PM-Scl,andJo1.我们也纳入了约翰.医院住院的曾行肌活检的抗Jo-1阳性的多发性肌炎（PM）患者。分析的病理改变包括束周萎缩，血管周围炎，线粒体功能障碍，原发炎症及肌纤维坏死。同时分析了病程，活检部位以及治疗。
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结果
共纳入91例皮肌炎和7例抗Jo-1阳性的PM患者。在单因素分析中，与相应抗体阴性的患者相比，TIF1-γ阳性患者有更多的线粒体功能障碍(47%vs18%;p=0.05)，NXP2+患者原发炎症反应较少(0%vs28%;p=0.01),Mi-2+患者原发炎症反应较多(50%vs19%;p=0.03),PM-Scl+患者原发炎症反应较多(67%vs18%;p=0.)。多因素分析显示TIF1-γ+患者线粒体功能障碍更明显（PR2.6,95%CI1.0–6.5,p=0.05），PM-Scl+患者原发炎症更明显(PR5.2,95%CI2.0–13.4;p=0.)，与活检时病程，活检部位，活检时的治疗无关，但因样本量较小，可靠性有限。诊断皮肌炎和多发性肌炎的抗Jo-1阳性的患者其肌活检无明显差异。
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结论
皮肌炎患者抗体表现不同具有不同的病理特点。合并与不合并皮疹的抗Jo-1阳性的患者肌活检表现无明显差异。
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附原文
Abstract：OBJECTIVE:Individualdermatomyositis(DM)-associatedautoantibodiesareassociatedwithdistinctclinicalphenotypes.Thisstudywasundertakentoexploretheassociationoftheseautoantibodieswithspecificmusclebiopsyfeatures.METHODS:DMsubjectswithamusclebiopsyreviewedatJohnsHopkinshadserascreenedforautoantibodiesrecognizingMi-2,transcriptionalintermediaryfactor1-γ(TIF1-γ),NXP2,MDA5,Ro52,PM-Scl,andJo1.Wealsoincludedanti-Jo1–positivepatientswithpolymyositis(PM)whohadabiopsyreadatJohnsHopkins.Analyzedhistologicalfeaturesincludedperifascicularatrophy,perivascularinflammation,mitochondrialdysfunction,primaryinflammation,andmyofibernecrosis.Durationofdisease,biopsylocation,andtreatmentatbiopsywerealsoanalyzed.RESULTS:Westudied91DMand7anti-Jo1–positivepatientswithPM.Inunivariateanalyses,TIF1-γ+patientshadmoremitochondrialdysfunction(47%vs18%;p=0.05),NXP2+patientshadlessprimaryinflammation(0%vs28%;p=0.01),Mi-2+patientshadmoreprimaryinflammation(50%vs19%;p=0.03),andPM-Scl+patientshadmoreprimaryinflammation(67%vs18%;p=0.)thanthosewhowerenegativeforeachautoantibody.Althoughreliabilitywaslimitedbecauseofsmallsamplenumbers,multivariateanalysisconfirmedthatTIF1-γ+patientshadmoremitochondrialdysfunction[prevalenceratio(PR)2.6,95%CI1.0–6.5,p=0.05]andPM-Scl+patientshadmoreprimaryinflammation(PR5.2,95%CI2.0–13.4;p=0.)independentofdiseasedurationatbiopsy,biopsysite,andtreatmentatbiopsy.Nodifferencesinmusclebiopsyfeatureswerenotedbetweenanti-Jo1–positivepatientsdiagnosedwithDMandPM.CONCLUSION:TheprevalenceofdifferenthistologicalfeaturesvariesaccordingtoautoantibodystatusinDM.Musclebiopsyfeaturesaresimilarinanti-Jo1patientswithandwithoutarash.
6
引自
Pinal-FernandezI,Casciola-RosenLA,Christopher-StineL,etal.ThePrevalenceofIndividualHistopathologicFeaturesVariesaccordingtoAutoantibodyStatusinMuscleBiopsiesfromPatientswithDermatomyositis.JRheumatol.Aug;42(8):-54.
