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

Research output: Contribution to journal › Article › peer-review

65 Citations (SciVal)

556 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 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

Keywords

Journal Article

Access to Document

10.3899/jrheum.160871

jrheum.160871.fullFinal published version, 802 KB
HMGCRJ Rheum revised version 2 clean version

Cite this

@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 = apr,

day = "1",

doi = "10.3899/jrheum.160871",

language = "English",

volume = "44",

pages = "488--492",

journal = "The Journal of Rheumatology",

issn = "0315-162X",

publisher = "Journal of Rheumatology",

number = "4",

}

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 - 2017/4/1

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

UR - https://www.scopus.com/pages/publications/85016930024

U2 - 10.3899/jrheum.160871

DO - 10.3899/jrheum.160871

M3 - Article

C2 - 28202739

SN - 0315-162X

VL - 44

SP - 488

EP - 492

JO - The Journal of Rheumatology

JF - The Journal of Rheumatology

IS - 4

ER -

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

Abstract

Keywords

Access to Document

Other files and links

Fingerprint

Cite this