Definition:

Henoch-Schonlein purpura nephritis (HSPN) is a rare kidney disease leading to chronic kidney disease in a non-negligible percentage of patients. Generally, the prognosis of Henoch-Schonlein purpura [DS:H01584] in children is favorable, but there are severe complications, one of which is renal involvement. HSPN is mainly characterized by acute episodes of glomerular inflammation with endocapillary and mesangial proliferation, fibrin deposits, and epithelial crescents that can heal spontaneously or lead to chronic lesions. HSPN and IgA nephropathy [DR:H01581] are currently considered related diseases resulting from glomerular deposition of aberrantly glycosylated IgA1. Although both nephritides present with similar histological findings and IgA abnormalities, they display pathophysiological differences with important therapeutic implications. Although there have been few evidence-based treatment strategies for HSPN, several studies have suggested that immunosuppressive drugs and multiple drug combination therapy were effective in ameliorating proteinuria and histological severity.