抗HMGCR抗体是一种在幼年特发性炎性肌

翻译者:高洁,医院临床免疫科

目的:

对英国青少年肌炎患者抗HMGCR的患病率进行队列研究。

方法:

对例青少年皮肌炎患者进行抗HMGCR酶联免疫吸附检测。

结果:

研究对象中有4例患者检测到抗HMGCR抗体(1%)。这些孩子有明显的肌肉症状,而没有或仅有轻微皮疹。肌肉活检有较特意表现,镜下可见广泛的纤维坏死及慢性炎症细胞浸润。这4例患者均存在肌酸激酶的长期升高,并且最终均接受生物制剂治疗。

结论:

抗HMGCR抗体在英国儿童肌炎患者中提示疾病严重程度高,且对常规治疗反应不佳。

参考文献:Anti-HMGCRAutoantibodiesinJuvenileIdiopathicInflammatoryMyopathiesIdentifyaRarebutClinicallyImportantSubsetofPatients

Objective.Weaimedtoestablishtheprevalenceandclinicalassociationsofanti-HMG-CoA-reductase(anti-HMGCR)inalargeUKcohortwithjuvenilemyositis.

Methods.Therewerepatientsinvestigatedforanti-HMGCRusingELISA.

Results.Anti-HMGCRautoantibodiesweredetectedin4patients(1%).Thesechildrenhadnoorminimalrashandsignificantmuscledisease.Musclebiopsieswereconsidereddistinctive,withwidespreadvariationinfibersize,necroticfibers,andchronicinflammatorycellinfiltrates;allhad

prolongedelevationofcreatinekinaseandallultimatelyreceivedbiologictherapies.

Conclusion.Anti-HMGCRinUKchildrenwithmyositisareassociatedwithseverediseasethatispoorlyresponsivetostandardtreatment.

JRheumatol;44;-/span

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