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Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children

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Abstract

Background

Several recent studies have shown improved short-term outcome of steroid-resistant nephrotic syndrome (SRNS) in children; however, only a few studies have evaluated the long-term outcome. The aims of our study were to obtain detailed data and analyze the long-term outcome of children with SRNS.

Methods

Sixty-nine children with idiopathic SRNS were enrolled and divided into two groups based on initial histopathological patterns: focal segmental glomerulosclerosis (FSGS) and minimal change (MC)/diffuse mesangial proliferation (DMP). The effects of initial treatment with the immunosuppressant of choice (cyclosporine or cyclophosphamide) on renal survival, remission, and incidence of complications were analyzed in both groups (4 subgroups).

Results

The renal survival rate was significantly different among the four different subgroups based on different combinations of initial histopathological pattern (FSGS vs. MC/DMP) and initial immunosuppressant used for treating SRNS (cyclosporine vs. cyclophosphamide) (P = 0.013), with renal survival in the FSGS (cyclophosphamide) subgroup being especially low (54.6 %). Disease- and/or treatment-associated complications were relatively low; however, hypertension at last examination was observed in a considerable number of patients (31.9 %).

Conclusions

Our results suggest that a recently developed therapeutic regimen with cyclosporine considerably improves both the initial remission rate and the long-term renal survival rate of children with idiopathic SRNS.

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Acknowledgments

The results presented in this paper have not been published previously in whole or part. The authors would like to thank Drs. Kentaro Ogata (Tokyo), Ryugo Hiramoto (Chiba), Takeshi Matsuyama (Tokyo), Hitoshi Wakaki (Tokyo), and Kaori Kikunaga (Tokyo) for their contributions to this study.

Financial declaration

Kenji Ishikura has received lecture fees from Novartis Pharma and Asahi Kasei Pharma. Yuko Hamasaki has received research grants from Novartis Pharma and lecture fees from Novartis Pharma, Astellas Pharma, and Pfizer Japan. Hiroshi Hataya has received lecture fees from Asahi Kasei Pharma, Astellas Pharma, Baxter, JMS, and Meiji Seika Pharma Co. Ltd. Masataka Honda has received lecture fees from Novartis Pharma, Takeda Pharmaceutical Co. Ltd., Chugai Pharmaceutical Co. Ltd., Japan Blood Products Organization, JCR Pharmaceuticals Co. Ltd., and Asahi Kasei Pharma. Masaaki Mori has received lecture fees from Astellas Pharma, Pfizer Japan, Asahi Kasei Pharma, Meiji Seika Pharma Co. Ltd., and Chugai Pharmaceutical Co. Ltd.

Ethical approval

The study was conducted in accordance with the ethical principles set out in the Declaration of Hesinki, and with the ethical guideline for epidemiological studies issued by the Minstry of Health, Labor and Welfare in Japan. The study was approved by the ethics committee of Tokyo Metropolitan Children's medical Center (H25-2).

Informed Consent

Because data were reported retrospectively based on patient charts, informed consent was not obtained, in accordance with the above guidelines.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Yokohama City University Medical Center, Kanagawa, Japan

    Aya Inaba & Masaaki Mori

  2. Department of Pediatric Nephrology, Toho University Faculty of Medicine, 6-11-1, Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan

    Yuko Hamasaki

  3. Department of Nephrology, Tokyo Metropolitan Children’s Medical Center, Tokyo, Japan

    Kenji Ishikura, Riku Hamada, Hiroshi Hataya & Masataka Honda

  4. Department of Pediatrics, Shiga University of Medical Science, Shiga, Japan

    Tomoyuki Sakai

  5. Community Health Welfare Division, Kawasaki Saiwai Ward Office Health and Welfare Center, Kanagawa, Japan

    Fumiyo Komaki

  6. Department of Clinical Research, Tokyo Metropolitan Children’s Medical Center, Tokyo, Japan

    Tetsuji Kaneko

  7. Department of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan

    Kenji Ishikura

  8. Teikyo Academic Research Center, Teikyo University, Tokyo, Japan

    Tetsuji Kaneko

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  1. Aya Inaba
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  2. Yuko Hamasaki
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Corresponding author

Correspondence to Yuko Hamasaki.

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Supplementary figure

The detailed clinical course of 9 patients who reached ESKD. Figure shows detailed clinical course of 9 patients who reached ESKD from the date when they were diagnosed as SRNS to the date when they reached ESKD. Cases 3 and 5 showed diffuse mesangial proliferation in the initial renal biopsy, and the other 7 cases showed focal segmental glomerulosclerosis. Alb Serum albumin (g/dl), Cr serum creatinine (mg/dl) (GIF 262 kb)

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Inaba, A., Hamasaki, Y., Ishikura, K. et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children. Pediatr Nephrol 31, 425–434 (2016). https://doi.org/10.1007/s00467-015-3174-7

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  • DOI: https://doi.org/10.1007/s00467-015-3174-7

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