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10.1002/acr.23113

http://scihub22266oqcxt.onion/10.1002/acr.23113
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suck abstract from ncbi


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pmid28129483
      Arthritis+Care+Res+(Hoboken) 2017 ; 69 (7 ): 1088-1094
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  • Association of Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme A Reductase Autoantibodies With DRB1*07:01 and Severe Myositis in Juvenile Myositis Patients #MMPMID28129483
  • Kishi T ; Rider LG ; Pak K ; Barillas-Arias L ; Henrickson M ; McCarthy PL ; Shaham B ; Weiss PF ; Horkayne-Szakaly I ; Targoff IN ; Miller FW ; Mammen AL
  • Arthritis Care Res (Hoboken) 2017[Jul]; 69 (7 ): 1088-1094 PMID28129483 show ga
  • OBJECTIVE: Autoantibodies 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. METHODS: The 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). RESULTS: Five 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. CONCLUSION: Compared 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.
  • |*Severity of Illness Index [MESH]
  • |Acyl Coenzyme A/*blood [MESH]
  • |Adolescent [MESH]
  • |Autoantibodies/*blood [MESH]
  • |Biomarkers/blood [MESH]
  • |Child [MESH]
  • |Child, Preschool [MESH]
  • |Female [MESH]
  • |Humans [MESH]
  • |Male [MESH]
  • |Myositis/*blood/*diagnosis [MESH]
  • |Nerve Tissue Proteins/*blood [MESH]


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