Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients
Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients
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DOI：
10.3899/jrheum.160871
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发表时间：
2017-04-01
影响因子：
3.9
通讯作者：
McHugh, Neil J.
中科院分区：
文献类型：
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作者：
Tansley, Sarah L.;Betteridge, Zoe E.;McHugh, Neil J.
Objective.我们的目的是在英国一个大型青少年肌炎队列中建立抗HMG-CoA还原酶（抗HMGCR）的患病率和临床相关性。采用ELISA法检测381例患者的抗HMGCR。4例患者（1%）检测到抗HMGCR自身抗体。这些儿童没有或只有轻微皮疹和严重的肌肉疾病。肌肉活检被认为是独特的，广泛的变化，在纤维大小，坏死纤维，慢性炎症细胞浸润，所有的肌酸激酶长期升高，并最终接受生物治疗。英国儿童肌炎患者的抗HMGCR与标准治疗反应不良的严重疾病相关。
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.