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

21 Citations (Scopus)
179 Downloads (Pure)

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 languageEnglish
Article numberjrheum.160871
JournalThe Journal of Rheumatology
Volume44
Issue number1
DOIs
Publication statusPublished - 15 Feb 2017

Fingerprint

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.
@article{6818ebb2d46b41009e4c784df992519f,
title = "Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients",
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 = "Journal Article",
author = "Tansley, {Sarah L} and Betteridge, {Zoe E} and Stefania Simou and Jacques, {Thomas S} and Clarissa Pilkington and Mark Wood and Kishore Warrier and Wedderburn, {Lucy R} and McHugh, {Neil J}",
year = "2017",
month = "2",
day = "15",
doi = "10.3899/jrheum.160871",
language = "English",
volume = "44",
journal = "The Journal of Rheumatology",
issn = "0315-162X",
publisher = "Journal of Rheumatology",
number = "1",

}

TY - JOUR

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 - 2017/2/15

Y1 - 2017/2/15

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

VL - 44

JO - The Journal of Rheumatology

JF - The Journal of Rheumatology

SN - 0315-162X

IS - 1

M1 - jrheum.160871

ER -