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Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients
- Source :
- Tansley, S L, Betteridge, Z E, Simou, S, Jacques, T S, Pilkington, C, Wood, M, Warrier, K, Wedderburn, L R & McHugh, N J 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. 4, pp. 488-492 . https://doi.org/10.3899/jrheum.160871
- Publication Year :
- 2016
-
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.
- Subjects :
- Male
Adolescent
Immunology
Anti-Inflammatory Agents
Enzyme-Linked Immunosorbent Assay
03 medical and health sciences
0302 clinical medicine
Rheumatology
Journal Article
Immunology and Allergy
Juvenile
Medicine
Humans
Child
Myositis
Autoantibodies
030203 arthritis & rheumatology
biology
business.industry
Standard treatment
Autoantibody
medicine.disease
Rash
Idiopathic inflammatory myopathies
Treatment Outcome
Child, Preschool
Cohort
biology.protein
Creatine kinase
lipids (amino acids, peptides, and proteins)
Female
Hydroxymethylglutaryl CoA Reductases
medicine.symptom
business
030217 neurology & neurosurgery
Subjects
Details
- ISSN :
- 0315162X
- Volume :
- 44
- Issue :
- 4
- Database :
- OpenAIRE
- Journal :
- The Journal of rheumatology
- Accession number :
- edsair.doi.dedup.....2b9f721456f2d61952176b4a8020c444