NATUREREVIEWS
NEUROLOGY
doi:10./nrneurol..44
Publishedonline22Apr
（中英）重症肌无力的
自身抗体特性及其对治疗的启示（一）
Abstract摘要
Myastheniagravis(MG)isanautoimmunedisordercausedbyautoantibodiesthattargettheneuromuscularjunction,leadingtomuscleweaknessandfatigability.Currentlyavailabletreatmentsforthediseaseincludesymptomaticpharmacologicaltreatment,immunomodulatorydrugs,plasmaexchange,thymectomyandsupportivetherapies.Differentautoantibodypatternsandclinicalmanifestationscharacterizedifferentsubgroupsofthedisease:early-onsetMG,late-onsetMG,thymomaMG,muscle-specifickinaseMG,low-densitylipoproteinreceptor-relatedprotein4MG,seronegativeMG,andocularMG.Thesesubtypesdifferintermsofclinicalcharacteristics,diseasepathogenesis,prognosisandresponsetotherapies.Patientswould,therefore,benefitfromtreatmentthatistailoredtotheirdiseasesubgroup,aswellasotherpossiblediseasebiomarkers,suchasantibodiesagainstcytoplasmicmuscleproteins.Here,wediscussthedifferentMGsubtypes,thesensitivityandspecificityofthevariousantibodiesinvolvedinMGfordistinguishingbetweenthesesubtypes,andthevalueofantibodyassaysinguidingoptimaltherapy.Anunderstandingoftheseelementsshouldbeusefulindetermininghowtoadaptexistingtherapiestotherequirementsofeachpatient.
重症肌无力是一种由自身抗体引起的自身免疫性疾病，其以神经肌肉接头为靶点，导致肌无力和肌疲劳。近来对此疾病的治疗方法包括药物对症治疗、免疫调节剂、血浆置换、胸腺切除术及支持疗法等。通过不同的自身抗体和临床表现可以将此疾病分为不同亚型：早发型MG、迟发型MG、胸腺瘤型MG、肌特异性激酶型MG、低密度脂蛋白受体相关蛋白4的MG、血清阴性型MG、眼肌型MG。这些亚型以临床特征，发病机制，预后，及其对治疗的反应而分类。因此，依据不同病人各自疾病亚型及其他可能的疾病生物学标记如抗细胞质肌蛋白抗体，实施对应的治疗方案，病人可能获益更多。这里，我们讨论不同的MG亚型，MG相关抗体的对应检测方法的敏感度和特异度，以及这些抗体检测结果用于指导最佳治疗方案的价值。理解这些因素有助于判定如何在现有治疗方案中选择对每个MG病人最合理的治疗。
Keypoints要点
Thecharacteristicmuscleweaknessinmyastheniagravis(MG)iscausedbyantibodiesdirectedagainsttheneuromuscularjunction
MGisdividedintosubgroupsonthebasisofspecificantibodies,otherbiomarkers,andclinicalcharacteristics,suchasageofonset,presenceofthymoma,andinvolvementofocularmuscles
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