Progress in IgA and the pathogenesis of Henoch-Schonlein purpura / 国际儿科学杂志

ABSTRACT

IgA plays an important role in the course of Henoch-Schonlein purpura.The current study shows that the HSP is characterized by IgA principally IgA1) depositions in the wall of dermal vessel and the renal mesangium, and IgA1 is deficient in galactose. IgA1 with aberrant glycosylation has a tendency to be self-aggregated, not to be efficiently cleared by the hepatic asialoglycoprotein receptor. It also weakens the ability of complement to remove immune complexes, and causes the exposure of new epitope and so on. All these lead to the formation of IgA1 immune complexes and the deposition in the wall of dermal vessel and the renal mesangium. IgA1 immune complexes activate complement principally through the alternative pathway, which causes inflammatory injury. In addition, various IgA autoantibodies, such as anti-endothelial cell antibodies,anticardiolipin antibodies and anti-neutrophil cytoplasmic antibodies, are also involved in the process of tissue damage of Henoch-Schonlein purpura.