In Focal Segmental Glomerulosclerosis (FSGS)
Elevated Proteinuria Increases Risk of Disease Progression
TARGET PROTEINURIA GOAL6,7,a
Achieving this goal correlates with extended kidney survival
aFSGS partial remission threshold defined as <1.5 g/g with a 40% reduction from baseline.
Significantly extended survival in patients with FSGS who achieved <1.5 g/g and 40% decrease in PCR7,b
Endpoints based on PCR follow-up 6 to 12 months from first nephrotic-range PCR valuec:
CR/FPRE-R, PCR <1.5 g/g and 40% decrease in PCR
FPRE-NR, not achieving CR/FPRE-R
Reducing proteinuria from nephrotic-range baseline to <1.5 g/g correlated with a 13-year extension in median survival probability vs patients who did not reach this thresholdc
CR=complete remission; eGFR=estimated glomerular filtration rate; ESKD=end-stage kidney disease; FPRE=FSGS partial remission endpoint; FPRE-NR=FPRE-non-responder; FPRE-R=FPRE-responder; FSGS=focal segmental glomerulosclerosis; PCR=protein-creatinine ratio; UP/C=urinary protein-to-creatinine ratio.
bTime to ESKD or death was analyzed using accelerated failure time modelling of the Weibull distribution and Kaplan-Meier estimates of cumulative incidence.
cBaseline pertains to first nephrotic-range proteinuria value (≥3.0 g/g) at or after disease onset.
Based on data from the National Registry of Rare Kidney Diseases (RaDaR), a UK Kidney Association initiative, in 270 patients with FSGS (150 adults age 18 and older and 120 pediatrics) with long-term data collection and comprehensive follow-up, representing a nationwide database and involving a large proportion of UK renal centers. ESKD was defined as chronic kidney disease stage 5 (confirmed eGFR <15 mL/min/1.73m2 or CKD stage 5 recorded in RaDaR) or receiving chronic dialysis or kidney transplant. Time to ESKD or death was analyzed using accelerated failure time modeling.
ACEi=angiotensin-converting enzyme inhibitor; ARB=angiotensin II receptor blocker; FDA=Food and Drug Administration; IgA=immunoglobulin A.
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