Clinicopathologic Changes in Children with Immunoglobulin A Nephritis and Henoch- Schönlein Purpura Nephritis after Cyclosporine A and Angiotensin-converting Enzyme Inhibitor Treatment

Lee, Jeong Ju; Kim, Yong-Jin; Shin, Jae Il; Yim, Hyunee; Park, Se Jin

doi:2013.17.2.92

J Korean Soc Pediatr Nephrol > Volume 17(2); 2013 > Article

Original 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.

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