ArticleArthritis care & research2017
Association of Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme A Reductase Autoantibodies With DRB1*07:01 and Severe Myositis in Juvenile Myositis Patients.
Article in Arthritis care & research, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 55 papers.
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Who cites it
55 citing papers in PubMed, 96 citations in OpenAlex.
- Juvenile Myositis: Insights on Classification, Pathogenesis, and Therapies Over the Past 50 Years.Arthritis care & research · 2026Review
- Clinical characteristics in antibody-positive pediatric patients with immune-mediated necrotizing myopathy.Italian journal of pediatrics · 2026Article
- Etiopathogenesis of Immune-Mediated Necrotizing Myopathy: A Comprehensive Review of Recent Advances.CNS neuroscience & therapeutics · 2026Review
- Review
- Natural products as HMG-CoA reductase inhibitors (statins) for the management of non-communicable diseases.Inflammopharmacology · 2026Review
- PROMIS displays strong construct validity in pediatric and adult patients with idiopathic inflammatory myopathies.Rheumatology (Oxford, England) · 2026Article
- Clinical-pathologic classification of anti-HMGCR-positive immune-mediated necrotizing myopathy.Scientific reports · 2025Article
- HLA loci heterozygosity modulates genetic risk in idiopathic inflammatory myopathies.Annals of the rheumatic diseases · 2025Article
- HMGCS1 variants cause rigid spine syndrome amenable to mevalonic acid treatment in an animal model.Brain : a journal of neurology · 2025Article
- Idiopathic Inflammatory Myopathies: Recent Evidence Linking Pathogenesis and Clinical Features.International journal of molecular sciences · 2025Review
- Precise identification and tracking of HMGCR-reactive CD4+ T cells in the target tissue of patients with anti-HMGCR immune-mediated necrotising myopathy.Annals of the rheumatic diseases · 2025Article
- Progressive Muscle Weakness: Identifying Necrotizing Autoimmune Myopathy as a Rare Culprit.Cureus · 2025Article
- Clinicopathological and imaging differences between pediatric and adult patients with anti-hydroxy-3-methyl-glutaryl-coenzyme A reductase necrotizing myopathy.Clinical rheumatology · 2025Review
- Neutrophil and mononuclear leukocyte pathways and upstream regulators revealed by serum proteomics of adult and juvenile dermatomyositis.Arthritis research & therapy · 2024Article
- Pediatric case of immune-mediated necrotizing myopathy with anti-HMGCR antibodies and dermatomyositis skin rash.Pediatric rheumatology online journal · 2024Article
- Absence of Pathogenic Mutations and Strong Association With HLA-DRB1*11:01 in Statin-Naïve Early-Onset Anti-HMGCR Necrotizing Myopathy.Neurology(R) neuroimmunology & neuroinflammation · 2024Article
- Advances in Juvenile Dermatomyositis: Pathophysiology, Diagnosis, Treatment and Interstitial Lung Diseases-A Narrative Review.Children (Basel, Switzerland) · 2024Review
- Myositis-Associated Autoantibodies in Patients With Juvenile Myositis Are Associated With Refractory Disease and Mortality.Arthritis & rheumatology (Hoboken, N.J.) · 2024Article
- Clinical Features and Immunogenetic Risk Factors Associated With Additional Autoantibodies in Anti-Transcriptional Intermediary Factor 1γ Juvenile-Onset Dermatomyositis.Arthritis & rheumatology (Hoboken, N.J.) · 2024Article
- Decreased dystrophin expression and elevated dystrophin-targeting miRNAs in anti-HMGCR immune-mediated necrotizing myopathy.Acta neuropathologica · 2023Article
Corrections and comments
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Authors and funding
13 authors at 9 institutions in 1 country.
Funding
Abstract
objectiveAutoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of juvenile anti-HMGCR-positive myositis patients.
methodsThe sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed. The features of anti-HMGCR-positive patients were compared to those of previously described adult patients with this autoantibody and to children with other myositis-specific autoantibodies (MSAs).
resultsFive of 440 patients (1.1%) were anti-HMGCR-positive; none had taken statin medications. Three patients had rashes characteristic of juvenile dermatomyositis and 2 patients had immune-mediated necrotizing myopathies. The median highest creatine kinase (CK) level of anti-HMGCR-positive subjects was 17,000 IU/liter. All patients had severe proximal muscle weakness, distal weakness, muscle atrophy, joint contractures, and arthralgias, which were all more prevalent in HMGCR-positive subjects compared to MSA-negative patients or those with other MSAs. Anti-HMGCR-positive patients had only partial responses to multiple immunosuppressive medications, and their disease often took a chronic course. The DRB1*07:01 allele was present in all 5 patients, compared to 26.25% of healthy controls (corrected P = 0.01); none of the 5 juvenile patients had DRB1*11:01.
conclusionCompared to children with other MSAs, muscle disease appears to be more severe in those with anti-HMGCR autoantibodies. Like adults, children with anti-HMGCR autoantibodies have severe weakness and high CK levels. In contrast to adults, in anti-HMGCR-positive children, there is a strong association with HLA-DRB1*07:01.
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