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

doi:10.3899/jrheum.160871

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

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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.

Original language	English
Pages (from-to)	488-492
Number of pages	5
Journal	The Journal of Rheumatology
Volume	44
Issue number	4
Early online date	1 Apr 2017
DOIs	https://doi.org/10.3899/jrheum.160871
Publication status	Published - 1 Apr 2017

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Journal Article

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T1 - Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients

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

PY - 2017/4/1

Y1 - 2017/4/1

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.

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.

KW - Journal Article

UR - https://doi.org/10.3899/jrheum.160871

U2 - 10.3899/jrheum.160871

DO - 10.3899/jrheum.160871

M3 - Article

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SN - 0315-162X

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JO - The Journal of Rheumatology

JF - The Journal of Rheumatology

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ER -

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

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