title: Childhood autoimmune necrotizing myopathy with anti-signal recognition particle antibodies.
abstract: BACKGROUND
  The 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).
  
  
  METHODS
  Before 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.
  
  
  RESULTS
  Clinical 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.
  
  
  CONCLUSIONS
  Childhood 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 Nerve 56: 1181-1187, 2017.
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing, 100034, China.
authors.forename: Yawen
authors.initials: Y
authors.lastname: Zhao
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing, 100034, China.
authors.forename: Xiujuan
authors.initials: X
authors.lastname: Liu
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing, 100034, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Zhang
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing, 100034, China.
authors.forename: Yun
authors.initials: Y
authors.lastname: Yuan
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood* / Q000000981:diagnostic imaging* / Q000188:drug therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D009135:Muscular Diseases / Q000097:blood* / Q000000981:diagnostic imaging* / Q000188:drug therapy; D009336:Necrosis / Q000097:blood / Q000000981:diagnostic imaging / Q000188:drug therapy; D018271:Signal Recognition Particle / Q000097:blood*
chemical_list: D001323:Autoantibodies; D007155:Immunologic Factors; D018271:Signal Recognition Particle
keywords: autoimmune necrotizing myopathy with anti-signal recognition particle antibodies; childhood; immune-modulation therapy; muscle edema; thigh MRI