Pathogenesis of galactose-deficient immunoglobulin A1 in children with Henoch-Sch?nlein purpura nephri-tis / 国际儿科学杂志

ABSTRACT

Henoch-Sch?nlein purpura ( HSP ) is a well-known systemic vasculitis disease mediated by IgA1 immune complex in children, and the pathogenesis remains unclear. Henoch-Sch?nlein purpura nephritis ( HSPN) is the most serious complication when the kidneys are damaged. It is also the key to determine the prog-nosis of the disease. Due to a combination of genetic,environmental,and infectious factors,IgA1 is abnormally glycosylated during the immune response. Galactose-deficient immunoglobulin A1(Gd-IgA1)is easy to self-ag-gregate and recognized by the antibody to form an immune complex deposited in the glomerular mesangial, which lead to kidney damage. This article reviews the progress of a series of pathogenic mechanisms of IgA1 glycosylation abnormality in HSPN.