HDCN: Review of abstract by Bergstein et al. Response of <b> crescentic Henoch-Schoenlein purpura nephritis </b> (HSP GN) to corticosteroid and azathioprine (AZA) therapy (1996 ASN Meeting)

Bergstein J, Leiser J, Andreoli S
Response of crescentic Henoch-Schoenlein purpura nephritis (HSP GN) to corticosteroid and azathioprine (AZA) therapy
Am Soc Nephrol
J Am Soc Nephrol (abstract) (Sep) 7:1328 1996

Henoch-Schoenlein Purpura (HSP) is a common childhood vasculitis that affects numerous organ systems, including the skin, joints, gastrointestinal tract and kidneys. Renal involvement may range from mild asymptomatic hematuria and/or proteinuria to rapidly progressive GN with or without the nephrotic syndrome. Children with the most severe renal involvement are at risk for the development of chronic renal failure. Although many different immunosuppressive regimens have been attempted for severe HSP renal disease, none has been proven to be effective, and most pediatric nephrologists base their treatment decisions on personal experience and/or anecdotal reports.

In this study, the authors report their experience with using azathioprine and corticosteroids to treat children with severe HSP renal disease. Over an 18 year period, they have seen 76 children with HSP-related GN. 30 of these children underwent renal biopsy because of either heavy proteinuria, renal insufficiency, or a "nephritic and/or nephrotic onset." 19 children had crescents on biopsy and were treated. Initial treatment consisted of daily azathioprine plus either high-dose daily steroids for 8 weeks or 6 pulses of IV steroids. This was then followed by every-other day steroids plus daily azathioprine for an average of 15 months. Average length of follow-up was 32 months.

Treated children did well: at last follow-up, 16/19 had resolved their hematuria, all had normal renal function (average creatinine clearance 122 +/- 26 ml/min/1.73m2) and proteinuria was markedly reduced from 8.8 +/- 7.5 g/day to 0.5 +/- 0.4 g/day. These results were statistically significant when compared to the patients' status prior to treatment. The authors conclude that long-term treatment with azathioprine and corticosteroids may be effective therapy for crescentic HSP GN.

Comment: The point that there is no agreed-upon treatment for severe HSP GN is highlighted by the fact that there were two papers presented at this meeting with different approaches to similar patients. Flynn et al report that treatment with oral cyclophosphamide was effective in six children with HSP-related nephrotic syndrome, most of whom also had crescents on renal biopsy. While both of these studies demonstrate a beneficial effect of the treatments used, neither study was very large and neither study included a control group of untreated patients. We must therefore conclude that the optimal approach to this disease is still unknown. (Joseph Flynn, M.D., University of Michigan, Ann Arbor)

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Am Soc Nephrol
Proteinuria/Hematuria : Henoch-Schoenlein Purpura