title: Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients.
abstract: We aimed to establish the prevalence and clinical associations of anti-HMG-CoA-reductase (anti-HMGCR) in a large UK cohort with juvenile myositis.
  
  
  
  There were 381 patients investigated for anti-HMGCR using ELISA.
  
  
  
  Anti-HMGCR autoantibodies were detected in 4 patients (1%). These children had no or minimal rash and significant muscle disease. Muscle biopsies were considered distinctive, with widespread variation in fiber size, necrotic fibers, and chronic inflammatory cell infiltrates; all had prolonged elevation of creatine kinase and all ultimately received biologic therapies.
  
  
  
  Anti-HMGCR in UK children with myositis are associated with severe disease that is poorly responsive to standard treatment.
authors.forename: Sarah L
authors.initials: SL
authors.lastname: Tansley
authors.forename: Zoe E
authors.initials: ZE
authors.lastname: Betteridge
authors.forename: Stefania
authors.initials: S
authors.lastname: Simou
authors.forename: Thomas S
authors.initials: TS
authors.lastname: Jacques
authors.forename: Clarissa
authors.initials: C
authors.lastname: Pilkington
authors.forename: Mark
authors.initials: M
authors.lastname: Wood
authors.forename: Kishore
authors.initials: K
authors.lastname: Warrier
authors.forename: Lucy R
authors.initials: LR
authors.lastname: Wedderburn
authors.forename: Neil J
authors.initials: NJ
authors.lastname: McHugh
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D006903:Hydroxymethylglutaryl CoA Reductases / Q000276:immunology*; D008297:Male; D009220:Myositis / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; C553220:HMGCR protein, human; D006903:Hydroxymethylglutaryl CoA Reductases
keywords: AUTOANTIBODIES; PEDIATRIC DERMATOMYOSITIS/POLYMYOSITIS; PEDIATRIC RHEUMATIC DISEASES; PROGNOSIS