Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients

作者：Tansley Sarah L; Betteridge Zoe E; Simou Stefania; Jacques Thomas S; Pilkington Clarissa; Wood Mark; Warrier Kishore; Wedderburn Lucy R; McHugh Neil J

来源：Journal of Rheumatology, 2017, 44(4): 488-492.

DOI：10.3899/jrheum.160871

• PEDIATRIC DERMATOMYOSITIS/POLYMYOSITIS
• PEDIATRIC RHEUMATIC DISEASES
• AUTOANTIBODIES
• PROGNOSIS

Objective. We aimed to establish the prevalence and clinical associations of anti-HMG-CoA-reductase (anti-HMGCR) in a large UK cohort with juvenile myositis.
Methods. There were 381 patients investigated for anti-HMGCR using ELISA.
Results. 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.
Conclusion. Anti-HMGCR in UK children with myositis are associated with severe disease that is poorly responsive to standard treatment.

• 出版日期2017-4