Henoch-Schönlein Nephritis: A Nationwide Study

Abstract

Background/Aim: The aim of this retrospective study was to evaluate the presentation, clinical and pathological manifestations and outcome of the Henoch-Schönlein purpura (HSP) nephritis in children. Methods: Clinical and laboratory data of 443 children with HSP nephritis aged between 3 and 16 years from 16 pediatric nephrology reference centers were analyzed retrospectively. The biopsy findings were graded according to the classification developed by the International Study of Kidney Disease in Children (ISKDC). Results: Renal biopsy was performed in 179 of the patients with HSP nephritis. The most common presenting clinical finding in patients who were biopsied was nephrotic range proteinuria (25%) which was followed by nephritic-nephrotic syndrome (23.5%). The biopsy findings according to the ISKDC were as follows: class I: 8.3%; II: 44.1%; III: 36.3%; IV: 6.7%; V: 3.3%; VI: 1.1%. All of the patients who developed end-stage renal disease had nephritic-nephrotic syndrome at presentation. Of 443 patients, 87.2% had a favorable outcome and 12.8% had an unfavorable outcome. The overall percentage of children who developed end-stage renal disease at follow-up was 1.1%. Logistic regression analysis did not show any association of initial symptoms and histology with outcome. Conclusion: In the presented cohort, the presence of crescents in the first biopsy or presenting clinical findings did not seem to predict the outcome of HSP nephritis in children. We conclude that children with HSP nephritis even with isolated microscopic hematuria and/or mild proteinuria should be followed closely.

The incidence and severity of Henoch-Schönlein purpura nephritis over a 22-year period in Fukushima Prefecture, Japan

“…However, all of the reports were retrospective studies. In a review of these reports, Zaffanello et al stated that the treatment of HSPN has not been adequately guided by evidence obtained in properly designed, randomized, placebocontrolled trials with outcome markers related to the progression to end stage renal disease and suggested that multicenter and randomized control studies should be undertaken in order to obtain results with good statistical power [22]. As to the clinical stage at 2 years after treatment, 13% of patients in Group 1 and 3% in Group 2 had active renal disease, and 2% in Group 1 and none patients in Group 2 had renal insufficiency.…”

Henoch–Schönlein purpura

“…In general, patients with microscopic hematuria and trivial proteinuria have an excellent prognosis [32,33,34 ]. By contrast, patients with nephritis complicated by nephrotic syndrome have a poor prognosis, with 20-40% of such patients experiencing long-term renal impairment [32,33,34 ].…”

“…In general, patients with microscopic hematuria and trivial proteinuria have an excellent prognosis [32,33,34 ]. By contrast, patients with nephritis complicated by nephrotic syndrome have a poor prognosis, with 20-40% of such patients experiencing long-term renal impairment [32,33,34 ]. The development of end stage renal disease is almost exclusively confined to patients with nephritis complicated by nephrotic syndrome [32,33,34 ].…”

“…By contrast, patients with nephritis complicated by nephrotic syndrome have a poor prognosis, with 20-40% of such patients experiencing long-term renal impairment [32,33,34 ]. The development of end stage renal disease is almost exclusively confined to patients with nephritis complicated by nephrotic syndrome [32,33,34 ]. Most studies showed a poor correlation between clinical features or histological features on biopsy with the ultimate outcome of HSP nephritis [32,33,34 ].…”

Henoch–Schönlein purpura and recurrent renal failure

“…Increasing mean proteinuria levels during follow-up were associated with progression. Soylemezoglu et al 6 in a study of 443 children with HSPN were unable to show any association between initial symptoms and histology with outcome. Finally, Rauta et al, 7 in an evaluation of 38 Finnish patients with HSPN noted no histopathological findings that were associated with poor outcome.…”