Challenging Clinical Practice Guideline Definitions in Childhood Nephrotic Syndrome.
A large Canadian cohort study found that children with steroid-resistant nephrotic syndrome who achieve remission have a similar CKD risk to steroid-sensitive children, and that the IPNA 2023 and KDIGO 2021 frequent-relapse definitions yield comparable relapse and CKD outcomes, supporting refined definitions and a 3-year relapse-free surrogate for long-term remission.
Background
In childhood nephrotic syndrome, definitions of immunosuppression treatment response, frequent relapses (FRNS), and long-term remission are conflicting and based on limited evidence. This study aimed to define these constructs based on their association with downstream disease outcomes rather than on consensus alone.
Key findings
The study included 1,114 children (median 3.8 years at diagnosis, 63% male, median 4.7-year follow-up) diagnosed between 1993 and 2023 in combined Canadian cohorts. Of these, 1,054 (95%) were steroid-sensitive and 60 (5%) steroid-resistant (SRNS), with 73% of SRNS achieving complete remission on steroid-sparing immunosuppression; no child with treatment-responsive SRNS developed CKD. Within one year of diagnosis, FRNS was classified in 27% of steroid-sensitive children by KDIGO 2021 and 36% by IPNA 2023 criteria, yet the two definitions produced similar relapse rates and CKD risk (2% each), though children meeting IPNA criteria less often received steroid-sparing immunosuppression. Ninety-eight percent of relapses occurred within three years of diagnosis or the last relapse.
Clinical implications
Because children with SRNS who achieve remission carry a CKD risk similar to steroid-sensitive children, the authors support defining treatment resistance by response to any immunosuppressive medication rather than to steroids alone. The comparable outcomes across IPNA 2023 and KDIGO 2021 frequent-relapse criteria support adopting the IPNA FRNS definition, and the concentration of relapses within three years supports using three-year relapse-free survival as a practical surrogate for long-term remission.
Category
Guidelines
Source
CJASN
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