TY - JOUR T1 - Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.160871 SP - jrheum.160871 AU - Sarah L. Tansley AU - Zoe E. Betteridge AU - Stefania Simou AU - Thomas S. Jacques AU - Clarissa Pilkington AU - Mark Wood AU - Kishore Warrier AU - Lucy R. Wedderburn AU - Neil J. McHugh Y1 - 2017/02/15 UR - http://www.jrheum.org/content/early/2017/02/28/jrheum.160871.abstract N2 - 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. ER -