Pathogenesis of galactose-deficient immunoglobulin A1 in children with Henoch-Schönlein purpura nephritis

Henoch-Schonlein purpura(HSP)is a well-known systemic vasculitis disease mediated by IgA1 immune complex in children, and the pathogenesis remains unclear.Henoch-Schonlein purpura nephritis(HSPN)is the most serious complication when the kidneys are damaged.It is also the key to determine the prognosis 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-aggregate 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. Key words: Children; Henoch-Schonlein purpura nephritis; Galactose-deficient IgA1; Immune complex; Pathogenesis