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我们对亨诺克-舒恩莱因紫癜发病机制的认识进展及其对改善其诊断的意义。

Advances in our understanding of the pathogenesis of Henoch-Schönlein purpura and the implications for improving its diagnosis.

机构信息

Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Suwon, Korea.

出版信息

Expert Rev Clin Immunol. 2013 Dec;9(12):1223-38. doi: 10.1586/1744666X.2013.850028.

DOI:10.1586/1744666X.2013.850028
PMID:24215411
Abstract

Henoch-Schönlein purpura (HSP) is a leukocytoclastic vasculitis classically characterized by palpable purpura, arthritis, abdominal pain and renal disease. In this article, we summarize our current understanding of the pathogenesis of HSP and the implications for improving its diagnosis. Although the pathogenesis of HSP is not fully understood yet, exciting new information has emerged in recent years, leading to a better understanding of its pathogenesis. Here, we discuss genetic predisposition, immunoglobulins with a particular emphasis on IgA1, activated complements, cytokines and chemokines, abnormal coagulation and autoantibodies in the underlying pathogenic mechanisms. Finally, diagnostic criteria for HSP developed by institutions such as the American College of Rheumatology and the European League against Rheumatism/Paediatric Rheumatology European Society were proposed to improve early detection and diagnosis.

摘要

过敏性紫癜(HSP)是一种白细胞碎裂性血管炎,其特征性表现为可触及性紫癜、关节炎、腹痛和肾脏疾病。本文总结了我们目前对 HSP 发病机制的认识及其对改善其诊断的意义。尽管 HSP 的发病机制尚未完全阐明,但近年来出现了令人兴奋的新信息,使我们对其发病机制有了更好的理解。在这里,我们讨论遗传易感性、免疫球蛋白(特别强调 IgA1)、激活的补体、细胞因子和趋化因子、异常凝血和自身抗体在潜在发病机制中的作用。最后,美国风湿病学会和欧洲抗风湿病联盟/儿科风湿病欧洲学会等机构提出了 HSP 的诊断标准,以提高早期检测和诊断。

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