Diagnosis and Treatment of Patients With Focal Segmental Glomerulosclerosis/Steroid-Resistant Nephrotic Syndrome: A Delphi Survey
Abstract
Focal segmental glomerulosclerosis (FSGS) is a histopathologic pattern of podocyte injury with several underlying etiologies and is characterized by segmental scarring that involves part of the glomerulus and a subset of glomeruli sampled on biopsy. In adults, nephrotic syndrome (NS) is often characteristic of primary (or idiopathic) FSGS.
In children, steroid-resistantnephrotic syndrome (SRNS) is an indication for kidney biopsy and most commonly associated with FSGS histologically. Available treatments do not always produce complete remission, and patients who do not achieve remission often progress to chronic kidney failure.
FSGS classification (primary, secondary, genetic, or undetermined cause) and patient-specific factors are used to individualize patient treatment in terms of medications used, dosing, and length of treatment. International guidelines are available to help nephrologists develop management strategies for patients with FSGS or SRNS, but the extent to which nephrologists agree with and may apply this guidance is unknown.
The Delphi FSGS and IgA Nephropathy Experts (DEFINE): Physicians study aimed to find consensus on pathophysiology, diagnosis, monitoring, and management of FSGS and IgA nephropathy among nephrologists from Canada, France, Germany, Italy, Spain, the United Kingdom, and the United States. In this 2-round online Delphi survey, agreement with 22 statements about FSGS/SRNS was scored by adult and pediatric nephrologists using a 1 to 9 Likert scale (9 = strongly agree).
Moderate versus high consensus were defined as 75% to 89% versus >=90% of participants scoring 7 to 9 on the Likert scale, respectively. Between November 2020 and April 2021, 207 nephrologists completed round 1, and 158 (76%) nephrologists completed round 2 (Figure S1, Tables S1-S4).
Methods and participant characteristics are detailed in the Supplementary Materials.