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

Research output: Contribution to journalArticle

19 Citations (Scopus)

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.

LanguageEnglish
Article numberjrheum.160871
JournalThe Journal of Rheumatology
Volume44
Issue number1
DOIs
StatusPublished - 15 Feb 2017

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Hydroxymethylglutaryl CoA Reductases
Myositis
Autoantibodies
Muscles
Biological Therapy
Creatine Kinase
Exanthema
Enzyme-Linked Immunosorbent Assay
Biopsy
Therapeutics
Juvenile dermatomyositis

Keywords

  • Journal Article

Cite this

Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients. / Tansley, Sarah L; Betteridge, Zoe E; Simou, Stefania; Jacques, Thomas S; Pilkington, Clarissa; Wood, Mark; Warrier, Kishore; Wedderburn, Lucy R; McHugh, Neil J.

In: The Journal of Rheumatology, Vol. 44, No. 1, jrheum.160871, 15.02.2017.

Research output: Contribution to journalArticle

Tansley, SL, Betteridge, ZE, Simou, S, Jacques, TS, Pilkington, C, Wood, M, Warrier, K, Wedderburn, LR & McHugh, NJ 2017, 'Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients', The Journal of Rheumatology, vol. 44, no. 1, jrheum.160871. https://doi.org/10.3899/jrheum.160871
Tansley, Sarah L ; Betteridge, Zoe E ; Simou, Stefania ; Jacques, Thomas S ; Pilkington, Clarissa ; Wood, Mark ; Warrier, Kishore ; Wedderburn, Lucy R ; McHugh, Neil J. / Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients. In: The Journal of Rheumatology. 2017 ; Vol. 44, No. 1.
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