Outcome of Henoch-Schönlein purpura nephritis treated with long-term immunosuppression

Abstract

This retrospective study investigated the outcome of 27 children (19 male) with Henoch-Schönlein purpura nephritis (HSN) of International Study of Kidney Disease in Children (ISKDC) grade 3b or higher treated with long-term immunosuppressive therapy in a single centre over a 10-year period. The mean age at presentation was 9.7 years. The median estimated glomerular filtration rate (eGFR) was 91.3 ml/min per 1.73 m(2), with the median urine protein creatinine ratio (UP:UC) 556 mg/mmol. The treatment protocol comprised daily steroids and cyclophosphamide for 8-12 weeks followed by azathioprine and a reducing regimen of alternate-day steroids for 8-12 months. After a mean follow-up period of 7 years following presentation, 37% made a complete recovery, 40.7% had persistent proteinuria, 7.4% had persistent proteinuria and were on antihypertensive therapy and 14.8% had progressed to end-stage kidney failure (ESKF). Children with poor outcome were older at presentation (p 0.005), had more crescents (p 0.015) and had heavier proteinuria 6 months post initial biopsy (p 0.023). All of the four children with ESKF had nephrotic range proteinuria and greater than 50% crescents on initial biopsy. Despite long-term immunosuppression, the majority of children with HSN grade 3b or higher will have persistent renal abnormalities on long-term follow-up.

Henoch-Schönlein Purpura Nephritis in Children: A Review

“…Shenoy et al found 15% patients progressed to ESRD and persistent renal abnormality with daily steroid and cyclophosphamide for 8-12 weeks followed by azathioprine and alternate day steroid for 8-12 weeks in severe form of HSP nephritis. 41 Good results obtained from more aggressive therapeutic regimen consisted with 3 Methyl Prednisolone(MP) pulses followed by 6 months course of prednisolone, depyridamole and 3 month course of cyclophosphamide with >60% complete remission by Oner et al 42 Other reported studies with multi drug therapy supported this result. [43][44][45][46] The randomized controlled study by Kawasaki et al showed combination of MP + urokinase pulses with cyclophosphamide was more effective than MP+urokinase pulses without cyclophosphamide in patients with>50% crescents.…”

Henoch-Schönlein Purpura Nephritis

“…As already mentioned, crescents are much more often seen in HSPN than in IgAN, and their number is related to the severity of clinical signs and to the prognosis of HSPN in most series (16,17,(53)(54)(55)(56)(57)(58)(59)(60). They are frequently seen in association with capillary wall destruction and endocapillary cell proliferation (33).…”

Henoch-Schönlein Purpura Nephritis in Childhood

“…Renal involvement occurs less frequently in children than adulthood (Yang et al, 2005;Pillebout et al, 2002). The incidence of nephritis in patients with Henoch-Schönlein purpura has been reported to be 15-62% with an estimated annual incidence of 20.4 per 100,000 children (Gardner-Medwin et al, 2002;Shenoy, Bradbury, et al 2007;Bogdanovic, 2009). The overall incidence of Henoch-Schönlein purpura nephritis and the severity of HenochSchönlein purpura nephritis in patients between 1987 and 1997 were similar to those in children between 1998 and 2008 and the number of patients with severe Henoch-Schönlein purpura nephritis has not decreased (Kawasaki et al, 2010).…”