Henoch–Schönlein purpura nephritis with nephrotic‐range proteinuria: histological regression possibly associated with cyclosporin A and steroid treatment

Abstract

Objective: To clarify the therapeutic role of cyclosporin A (CyA) for patients with Henoch–Schönlein purpura nephritis (HSPN) showing nephrotic‐range proteinuria.

Methods: The clinical and histological findings of eight children (7.7±3.8 years), who were treated with CyA and prednisolone, were evaluated retrospectively. All underwent a renal biopsy before therapy, and six of the eight patients received a follow‐up biopsy after therapy.

Results: The histological grade of the International Study of Kidney Disease in Children (ISKDC) was improved in all six patients who received a follow‐up biopsy (pre‐therapy, four grade IIIa and two grade IIIb; post‐therapy, one grade I and five grade II) and it was statistically significant (p = 0.031). The activity index was significantly decreased after therapy (8.3±1.6 vs. 3.5±1.5, p = 0.031), and the chronicity index (0.5±0.5 vs. 0.7±1.0) and tubulointerstitial (TI) scores (1.5±1.3 vs. 0.8±1.6) did not change. There was a reduction in proteinuria from 3.2±2.3 to 0.1±0.1 g/m2/day (p = 0.008) and renal function remained normal in all patients after therapy. However, one patient showed CyA‐induced nephrotoxicity at a second biopsy. After an average follow‐up period of 3.8 years, six patients showed normal urine and renal function, and two showed minor urinary abnormalities.

Conclusion: This study suggests that CyA therapy is effective in reducing proteinuria, which is a known risk factor for the development of renal insufficiency in HSPN and may regress the renal pathology in patients with nephrotic‐range proteinuria.