Original Articles
Effective therapy for severe Henoch-Schonlein purpura nephritis with prednisone and azathioprine: A clinical and histopathologic study

https://doi.org/10.1067/mpd.2000.103448Get rights and content

Abstract

Objectives: To validate a scoring system to assess the severity of renal lesions and to correlate histology with clinical findings. We also examined the efficacy of treatment with prednisone (1 to 2 mg/kg/d) and azathioprine (1 to 2 mg/kg/d) for severe Henoch-Schonlein purpura (HSP) nephritis. Methods: Twenty patients were evaluated retrospectively. All underwent biopsy before treatment, and 13 underwent biopsy after therapy. We developed a scale based on glomerular, tubulointerstitial (TI), and vascular changes and assigned all specimens acuity, chronicity, and TI scores. The outcomes of 17 patients were compared with those of a historical control group. Results: Chronicity score at initial biopsy increased with increasing delay between onset of renal involvement and first biopsy (ρ = 0.55, P =.016) but did not progress after treatment was initiated. Both acuity (ρ = 0.57,P =.016) and TI (ρ = 0.69, P =.003) scores correlated with clinical severity at first biopsy. The TI score correlated negatively with serum albumin (ρ = –.60, P <.01). Significantly more patients in the study group than in the control group had a favorable outcome (15 [88%] of 17 vs 32 [54%] of 59, P =.011). Conclusions: Our scale reflects disease activity and highlights the importance of TI changes in severe HSP nephritis. Outcome comparisons indicate that early treatment with prednisone and azathioprine prevents progression of chronic changes and improves outcome. (J Pediatr 2000;136:370-5)

Section snippets

Patients

The charts of all 20 patients (12 boys) treated between 1965 and 1995 for severe HSP nephritis were reviewed. Patients ranged in age from 4 to 19 years (mean 10.5 ± SD 4.0). To be considered for treatment, patients had to have proteinuria of at least 1 g/24 h. There was one exception, treated for recurrent episodes of gross hematuria, but with <1 g/24 h proteinuria. Patients had to be treated for at least 1 month to be included in the outcome analysis. Basic demographic information and clinical

Histopathologic Evaluation

Biopsy data from all 20 patients were used in this portion of the analysis. The scores assigned by the 2 observers did not differ significantly. The interobserver 95% limits of agreement were –5.3 to 5.5 for the acuity score (mean difference 0.09) and –1.5 to 1.6 for the chronicity score (mean difference 0.06). These were deemed acceptable.

The acuity score of the first biopsy correlated with clinical severity (ρ = 0.57, P =.016). When Kruskal-Wallis was performed, the difference in acuity

DISCUSSION

Although the prognosis for most children with HSP nephritis is very good, an estimated 10% or more of those referred to specialized nephrology centers will have end-stage renal failure.4, 5, 8 Because many patients with severe nephritis are treated, the true natural history of the disease is not known, and no randomized controlled trials of treatment for HSP nephritis have been conducted. Although a prospective randomized trial would be ideal, it seems unlikely that such a study will be

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Reprint requests: Bethany J. Foster, MD, Montreal Children’s Hospital, McGill University, 2300 Tupper Ave, Montreal, QC, H3H 1P3, Canada.

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