Henoch–Schönlein purpura nephritis in children

Abstract

Henoch-Schönlein purpura (HSP) is the most common vasculitis in children, in whom prognosis is mostly dependent upon the severity of renal involvement. Nephritis is observed in about 30% of children with HSP. Renal damage eventually leads to chronic kidney disease in up to 20% of children with HSP nephritis in tertiary care centres, but in less than 5% of unselected patients with HSP, by 20 years after diagnosis. HSP nephritis and IgA nephropathy are 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. HSP nephritis 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. By contrast, IgA nephropathy normally presents with slowly progressive mesangial lesions resulting from continuous low-grade deposition of macromolecular IgA1. This Review highlights the variable evolution of similar clinical and histological presentations among paediatric patients with HSP nephritis, which constitutes a challenge for their management, and discusses the treatment of these patients in light of current guidelines based on clinical evidence from adults with IgA nephropathy.

Association of endothelial nitric oxide synthase gene polymorphism with the risk of Henoch-Schönlein purpura/Henoch-Schönlein purpura nephritis

“…The prognosis of HSP is mostly dependent upon the severity of renal involvement. 14 In this meta-analysis, we also study the risk of progression of HSP into HSPN, and we found that eNOS G894T and T786C TT gene polymorphism were not associated with the risk of patients with HSP developing into HSPN. However, the number of studies included in this meta-analysis was small, and more studies should be conducted in future.…”

Henoch-Schönlein Purpura Nephritis in Children: Prognosis and Treatment Experiences

“…These presentations are shown to be predictors of a poor outcome (21). Similar biopsy results at the onset of HSPN have a variable evolution (22), but the significance of the degree of proteinuria for nephritis outcomes has been validated several times (12,17). Still, the unknowns about HSPN pathophysiology make it difficult to find clues from basic research as to which type of treatment is best.…”

Rectal Adenocarcinoma As An Uncommon Cause of Immunoglobulin A Vasculitis (Henoch-Schönlein Purpura)

“…8 Signs in the renal biopsy of IgA vasculitis are the same as those of IgA nephritis. 9 The etiology of IgA vasculitis is yet to be determined; however, there are often precipitating factors, such as infectious diseases, a reaction to drugs or malignancy. 10 It was reported in previous epidemiological studies that the disease was more commonly encountered especially in the autumn and winter months, so upper respiratory tract infections were suspected to be the main triggering factors.…”