J Korean Soc Pediatr Nephrol > Volume 17(2); 2013 > Article
J Korean Soc Pediatr Nephrol 2013;17(2): 92-100. doi: https://doi.org/10.3339/jkspn.2013.17.2.92
Immunuglobulin A 신질환과 Henoch-Schnlein purpura 신질환을 가진 소아에서의 cyclosporine A와 angiotensin-converting enzyme inhibitor 치료의 임 상적, 병리학적 변화
이정주, 김용진, 신재일, 임현이, 박세진
1아주대학교 의과대학 소아청소년과
2영남대학교 의과대학 병리학과*
3연세대학교 의과대학 소아청소년과†
4아주대학교 의과대학 병리학과‡
Clinicopathologic Changes in Children with Immunoglobulin A Nephritis and Henoch- Schönlein Purpura Nephritis after Cyclosporine A and Angiotensin-converting Enzyme Inhibitor Treatment
Jeong Ju Lee, Yong-Jin Kim, Jae Il Shin, Hyunee Yim, Se Jin Park
1Department of Pediatrics, Ajou University Hospital, Ajou Universtiy School of Medicine, Suwon, Korea,
2Department of Pathology*, Yeungnam University College of Medicine, Daegu, Korea,
3Department of Pediatrics†, Severance Children’s Hospital, Yonsei University College of Medicine, Seoul, Korea,
4Department of Pathology‡, Ajou University Hospital, Ajou Universtiy School of Medicine, Suwon, Korea
Corresponding Author: Se Jin Park ,Tel: 031-219-5163, Fax: 031-219-5169, Email: fli018@hanmail.net
Received: September 27, 2013;  Accepted: October 14, 2013.
This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons. org/licenses/bync/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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ABSTRACT
Purpose: To investigate the clinicopathologic effects of cyclosporine A (CsA) in children with diseases characterized by mesangial immunoglobulin A deposits such as immunoglobulin A nephropathy (IgAN) and Henoch-Schönlein purpura nephritis (HSPN).

Methods:
We retrospectively reviewed the clinicopathologic outcomes of 54 children (IgAN, 36; HSPN, 18) treated with CsA. The starting dose of CsA was 5 mg/kg per day, and it was administered in 2 divided doses. The degree of proteinuria and pathologic changes in renal biopsies were evaluated before and after CsA treatment.

Results :
The mean protein to creatinine ratio decreased from 3.7±1.5 to 0.6±0.4 (P <0.001), and 32 (59.2%) children achieved complete remission of proteinuria after 1-year CsA treatment. Among the 54 children, 24 maintained normal renal function and 25 exhibited microscopic hematuria or proteinuria at the end of CsA treatment. In the HSPN group, 3 children whose initial biopsies indicated class IIIb disease showed class II disease on follow-up, and the follow-up biopsies of 2 children who had class II disease indicated the same class II disease. In the IgAN group, cortical tubular atrophy occurred in 1 child, and no child with IgAN had cortical interstitial fibrosis or tubular atrophy after 1-year CsA treatment. No significant complications were found in the children treated with CsA.

Conclusion :
Our findings indicate that CsA treatment is effective and beneficial in reducing massive proteinuria and preventing progression to end-stage renal failure in children with glomerular diseases characterized by IgA deposits, such as IgAN and HSPN, within 1 year of treatment.
Key words: IgA deposit | IgA nephropathy | Henoch-Schönlein purpura nephritis | cyclosporine A
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