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

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

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  • 1Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Suwon, Korea.


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.

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