Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria: Histological regression possibly associated with cyclosporin A and steroid treatment

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.