Advances in our understanding of the pathogenesis of Henoch-Schönlein purpura and the implications for improving its diagnosis.
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.
Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Suwon, Korea.
Se Jin
SJ
Park
Jin-Soon
JS
Suh
Jun Ho
JH
Lee
Jung Won
JW
Lee
Seong Heon
SH
Kim
Kyoung Hee
KH
Han
Jae Il
JI
Shin
D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D001777:Blood Coagulation; D003167:Complement Activation; D016207:Cytokines / Q000276:immunology*; D005060:Europe; D020022:Genetic Predisposition to Disease; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology; D007070:Immunoglobulin A / Q000235:genetics / Q000276:immunology*; D017410:Practice Guidelines as Topic; D014481:United States
D001323:Autoantibodies; D016207:Cytokines; D007070:Immunoglobulin A