Pathogenesis of Henoch-Schönlein purpura nephritis.
The severity of renal involvement is the major factor determining the long-term outcome of children with Henoch-Schönlein purpura (HSP) nephritis (HSPN). Approximately 40% children with HSP develop nephritis, usually within 4 to 6 weeks after the initial onset of the typical purpuric rashes. Although the pathogenetic mechanisms are still not fully delineated, several studies suggest that galactose-deficient IgA1 (Gd-IgA1) is recognized by anti-glycan antibodies, leading to the formation of the circulating immune complexes and their mesangial deposition that induce renal injury in HSPN.
Lau
Keith K
KK
Department of Pediatrics, McMaster University, 1200 Main Street West, HSC 3N27, Hamilton, Ontario, Canada. keithklou@gmail.com
Suzuki
Hitoshi
H
Novak
Jan
J
Wyatt
Robert J
RJ
D000936:Antigen-Antibody Complex / Q000097:blood; D002648:Child; D005690:Galactose / Q000172:deficiency / Q000276:immunology; D005920:Glomerular Mesangium / Q000276:immunology / Q000378:metabolism; D005922:Glomerulonephritis, IGA / Q000235:genetics / Q000276:immunology / Q000378:metabolism*; D006801:Humans; D011695:IgA Vasculitis / Q000235:genetics / Q000276:immunology / Q000378:metabolism*; D007105:Immune Complex Diseases; D007070:Immunoglobulin A / Q000235:genetics / Q000276:immunology / Q000378:metabolism*
D000936:Antigen-Antibody Complex; D007070:Immunoglobulin A; D005690:Galactose