Childhood autoimmune necrotizing myopathy with anti-signal recognition particle antibodies

被引:8
|
作者
Zhao, Yawen [1 ,2 ]
Liu, Xiujuan [1 ,2 ]
Zhang, Wei [1 ,2 ]
Yuan, Yun [1 ,2 ]
机构
[1] Peking Univ First Hosp, Dept Neurol, Beijing, Peoples R China
[2] Peking Univ, Hosp 1, Dept Neurol, Beijing 100034, Peoples R China
关键词
autoimmune necrotizing myopathy with anti-signal recognition particle antibodies; childhood; immune-modulation therapy; muscle edema; thigh MRI; HISTOPATHOLOGICAL FEATURES; AUTOANTIBODIES;
D O I
10.1002/mus.25575
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
IntroductionThe aim of this study was to describe the therapeutic effects of immunomodulatory therapy in 3 patients with childhood autoimmune necrotizing myopathy with anti-signal recognition particle antibodies (SRP-ANM). MethodsBefore treatment, data on clinical features, muscle pathology, and thigh MRIs were obtained. After definitive diagnoses, all 3 patients were treated with intravenous immunoglobulin and corticosteroids, and thigh MRIs were performed. ResultsClinical improvements were associated with declines in serum creatine kinase levels. Pretreatment thigh MRIs revealed diffuse, but uneven or focal edema, mostly in the posterior thigh muscles, which was alleviated after immunomodulatory therapy. DiscussionChildhood SRP-ANM responded well to immunomodulatory therapy. The extent of edema, as monitored by thigh MRI, appears to be a useful marker of disease severity and therapeutic benefit. Muscle Nerve56: 1181-1187, 2017
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页码:1181 / 1187
页数:7
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