Abstract: FR-PO658
Anti-Nephrin Antibodies in Idiopathic Nephrotic Syndrome in Japanese Children
Session Information
- Pediatric Nephrology - II
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1900 Pediatric Nephrology
Authors
- Horinouchi, Tomoko, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Watts, Andrew James baxter, Department of Medicine/Renal Division, Brigham and Women’s Hospital, and Harvard Medical School, Boston, Massachusetts, United States
- Nagano, China, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Ichikawa, Yuta, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Inoki, Yuta, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Tanaka, Yu, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Kitakado, Hideaki, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Ueda, Chika, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Kondo, Atsushi, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Sakakibara, Nana, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Shima, Yuko, Department of Pediatrics, Wakayama Medical University, Wakayama, Japan
- Nozu, Kandai, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
- Sampson, Matt G., Department of Medicine/Renal Division, Brigham and Women’s Hospital, and Harvard Medical School, Boston, Massachusetts, United States
- Weins, Astrid, Department of Pathology, Brigham and Women’s Hospital, and Harvard Medical School, Boston, Massachusetts, United States
- Iijima, Kazumoto, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan
Background
Many patients with childhood idiopathic nephrotic syndrome are steroid-sensitive, suggesting the involvement of the immune system in the pathogenesis. Several genome-wide association studies have suggested a polygenic contribution, particularly in the HLA DR/DQ region and a locus including NPHS1, but the etiology remains unclear. Anti-nephrin antibodies have recently been reported in both adults and children with biopsy proven minimal change disease (MCD), but the presence of anti-nephrin antibodies in Japanese childhood idiopathic nephrotic syndrome (INS) has not been investigated.
Methods
Anti-nephrin antibodies were measured by ELISA in paired plasma samples obtained from 14 Japanese pediatric patients with INS (male/female: 8/6), at initial disease onset (active disease) and following steroid monotherapy. Clinical characteristics were compared between the anti-nephrin antibodies positive and negative groups.
Results
The median age at the onset was 75.5 months (interquartile range (IQR): 45-113). Steroid sensitivity resulted in complete remission in 13 patients and almost complete remission in one patient after 4 weeks of glucocorticoid monotherapy. Circulating anti-nephrin antibodies were detected in seven of 14 patients during active disease. In all cases, anti-nephrin antibodies were significantly reduced following treatment concordant with clinical response. There were no differences between the positive and negative groups in pre-treatment parameters. Of the 13 patients who achieved complete remission, nine had at least one relapse during a median follow-up of 851 days (IQR: 808-973). There was also no significant difference in the relapse-free period after the onset between the two groups (P=0.658).
Conclusion
We have identified circulating anti-nephrin antibodies at initial presentation in half of Japanese pediatric INS, which is a higher proportion than previously reported for a North American cohort of adults and children with biopsy proven MCD. Further studies are needed to establish the prognostic implications of anti-nephrin antibodies in childhood INS and the relationship with the NPHS1 risk variants in this population.
Funding
- Government Support – Non-U.S.