PT  - JOURNAL ARTICLE
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
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.