Clinical Decision-Making About Immunosuppressive Treatment in Focal Segmental Glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS) is a heterogeneous disorder with a high risk of progression to kidney failure. There are no approved therapies for FSGS, and futility of treatment is poorly defined. The Cure Glomerulonephropathy (CureGN) study offers the opportunity to describe the characteristics of participants who started immunosuppressive therapy (IST), never received IST, or in whom this treatment was discontinued.

An observational cohort.

Participants enrolled in CureGN with FSGS and surveyed nephrologists.

The clinical and laboratory data from participants with FSGS who were enrolled in the CureGN observational cohort were reviewed to define features associated with withholding initial IST or terminating ongoing IST. Nephrologists were surveyed about what factors would influence their decision to prescribe or withdraw IST in patients with FSGS.

(1) Identify factors associated with IST initiation and discontinuation in individuals with FSGS; and (2) Identify clinical and laboratory features nephrologists consider when they recommend against the use of IST at diagnosis (initiation of care) and during the course of disease.

Based on quantitative findings from the CureGN cohort and survey responses from practicing nephrologists, a low estimated glomerular filtration rate at presentation, significant glomerulosclerosis, and interstitial fibrosis and tubular atrophy on kidney biopsy make initiation of IST less likely.

Heterogeneous nature of the cohort and an inability to divide the patients into KDIGO subgroups of FSGS. Rationale for decision to stop or defer treatment was not available. More surveys were completed by pediatric providers, and the majority were completed by academic practitioners.

The factors that impact decisions about IST initiation and discontinuation were consistent among pediatric and internal medicine nephrologists, namely advanced scarring and lower estimated glomerular filtration rate. We suggest that this information should be incorporated into patient management guidelines and clinical trial design.