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.


CONCLUSIONS
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.
Shin
J I
JI
Department of Paediatrics, The Institute of Kidney Disease, Yonsei University College of Medicine, Severance Hospital, Seoul, Korea.
Park
J M
JM
Shin
Y H
YH
Kim
J H
JH
Lee
J S
JS
Jeong
H J
HJ
D000293:Adolescent; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D007668:Kidney / Q000473:pathology; D008297:Male; D009393:Nephritis / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D009401:Nephrosis / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage*; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D012074:Remission Induction; D012189:Retrospective Studies; D012720:Severity of Illness Index
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone