PT - JOURNAL ARTICLE AU - Tansley, Sarah L. AU - Betteridge, Zoe E. AU - Simou, Stefania AU - Jacques, Thomas S. AU - Pilkington, Clarissa AU - Wood, Mark AU - Warrier, Kishore AU - Wedderburn, Lucy R. AU - McHugh, Neil J. TI - Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients AID - 10.3899/jrheum.160871 DP - 2017 Apr 01 TA - The Journal of Rheumatology PG - 488--492 VI - 44 IP - 4 4099 - http://www.jrheum.org/content/44/4/488.short 4100 - http://www.jrheum.org/content/44/4/488.full SO - J Rheumatol2017 Apr 01; 44 AB - 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.