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. 2017 Apr;44(4):488-492.
doi: 10.3899/jrheum.160871. Epub 2017 Feb 15.

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

Sarah L Tansley, Zoe E Betteridge, Stefania Simou, Thomas S Jacques, Clarissa Pilkington, Mark Wood, Kishore Warrier, Lucy R Wedderburn, Neil J McHugh; Juvenile Dermatomyositis Research Group
Free article

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

Sarah L Tansley et al. J Rheumatol. 2017 Apr.
Free article

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Abstract

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.

Keywords: AUTOANTIBODIES; PEDIATRIC DERMATOMYOSITIS/POLYMYOSITIS; PEDIATRIC RHEUMATIC DISEASES; PROGNOSIS.

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