Advances in our understanding of the pathogenesis of Henoch-Schönlein purpura and the implications for improving its diagnosis. (December 2013)

• Record Type: Journal Article
• Authors: Park, Se Jin; Suh, Jin-Soon; Lee, Jun Ho; Lee, Jung Won; Kim, Seong Heon; Han, Kyoung Hee; Shin, Jae Il
• Is Part Of: Expert review of clinical immunology. Volume 9:Number 12(2013)
• Page Start: 1223
• Page End: 1238
• Publication Date: 2013-12
• Subjects: cytokines -- diagnosis -- genetic susceptibility -- Henoch-Schönlein purpura -- immunoglobulins -- pathogenesis -- vasculitis; Clinical immunology -- Periodicals; 616.079
• DOI: 10.1586/1744666X.2013.850028
• Languages: English
• ISSNs: 1744-666X

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.