A national survey of pediatric nephrologists on the treatment of steroid-resistant nephrotic syndrome.

Abstract

Aim: Steroid-resistant nephrotic syndrome (SRNS) in children is a common acquired cause of kidney failure. The treatment practices vary widely. With this web-based survey, we aim to report common medication and follow-up practices by pediatric nephrologists in the USA who treat children with SRNS.

Materials and methods: This survey was conducted via the Pediatric Nephrology Research Consortium (PNRC) and sent to 190 members of the PNRC between April 2017 and September 2018.

Results: 127 (67%) of the 190 member pediatric nephrologists of the PNRC responded to the survey. 46.5% of respondents were 35 - 44 years of age. Median number of years since fellowship training was 11.5 Definition of SRNS varied by number of weeks of resistance to steroids with 40% respondents using a 6-week cut-off, with others using a 4-week (28%) or 8-week (31%) cut-off. The majority (83%) of respondents use calcineurin inhibitors (CNI) as their first-line agent for steroid-resistant nephrotic syndrome. The majority (88%) of respondents use CNI if focal segmental glomerulosclerosis (FSGS) is present on the biopsy, while 63% of respondents use CNI if minimal change disease is present on the biopsy. There is significant variability in length of treatment with CNI for assessing response. The weaning schedule for CNI and steroids varies from weaning within weeks to months. 49% of respondents use rituximab if there is no response to CNI, and 29% use mycophenolate. Genetic testing in SRNS patients is pursued by most respondents.

Conclusion: This survey result highlights the common use of CNI in SRNS and various treatment approaches by a sizeable number of pediatric nephrologists in the USA.