Pathogenesis of Henoch-Schönlein purpura nephritis

Pediatr Nephrol. 2010 Jan;25(1):19-26. doi: 10.1007/s00467-009-1230-x. Epub 2009 Jun 13.

Abstract

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antigen-Antibody Complex / blood
  • Child
  • Galactose / deficiency
  • Galactose / immunology
  • Glomerular Mesangium / immunology
  • Glomerular Mesangium / metabolism
  • Glomerulonephritis, IGA / genetics
  • Glomerulonephritis, IGA / immunology
  • Glomerulonephritis, IGA / metabolism*
  • Humans
  • IgA Vasculitis / genetics
  • IgA Vasculitis / immunology
  • IgA Vasculitis / metabolism*
  • Immune Complex Diseases
  • Immunoglobulin A / genetics
  • Immunoglobulin A / immunology
  • Immunoglobulin A / metabolism*

Substances

  • Antigen-Antibody Complex
  • Immunoglobulin A
  • Galactose