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Scand J Rheumatol. 2005 Sep-Oct;34(5):392-5.

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

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1
Department of Paediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine, Severance Hospital, Seoul, Korea.

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.

PMID:
16234188
DOI:
10.1080/03009740510026544
[Indexed for MEDLINE]
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