Henoch-Schönlein
purpura (HSP) is a systemic disorder characterized by
leukocytoclastic vasculitis involving the capillaries and the deposition of
IgA immune complexes. Renal involvement is the principal cause of morbidity and mortality in children with HSP. Thus, it is important to clarify the onset mechanism as well as the prognostic factors of Henoch-Schönlein
purpura nephritis (HSPN) and to identify the most appropriate treatment. We herein review the pathogenesis, the prognostic factors and treatment of HSPN. As to the pathogenesis, several studies suggest that
galactose-deficient IgA1 (Gd-IgA1) is recognized by anti-
glycan antibodies, leading to the formation of circulating
immune complexes and their mesangial deposition, thereby inducing renal injury. With regard to the prognostic factors, a number of factors have been suggested including
nephrotic syndrome, decreased
factor XIII activity,
hypertension, severe renal injury, high renal accumulation of activated macrophage, alpha-smooth muscle actin, and high serum myeloid-related
protein levels. For the treatment of severe HSPN, aggressive
therapies including multiple
drug combination therapy and
plasmapheresis have been shown to be effective in ameliorating
proteinuria and histological severity. Nevertheless, detailed investigation into the pathogenesis of HSPN and double-blind randomized control studies on children with HSPN are still necessary.