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Kidney Week

Abstract: FR-PO1133

Idiopathic FSGS: Outcome in Adult Patients with Initial Steroid-Resistance

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Rood, Ilse M., Radboud University Medical Center, Nijmegen, Gelderland, Netherlands
  • Bavinck, Aernoud, Radboud University Medical Center, Nijmegen, Gelderland, Netherlands
  • van Rijn, Marieke, Radboud University Medical Center, Nijmegen, Gelderland, Netherlands
  • Deegens, Jeroen, Radboud University Medical Center, Nijmegen, Gelderland, Netherlands
  • Wetzels, Jack F., Radboud University Medical Center, Nijmegen, Gelderland, Netherlands
Background

Idiopathic focal segmental glomerulosclerosis (iFSGS) is a common cause of nephrotic syndrome (NS) in adults. Guidelines advise initial therapy with corticosteroids (CS). The clinical outcome of patients with initial steroid-resistance is ill-defined.

Methods

Retrospective cohort study of adult patients with biopsy-proven FSGS, nephrotic syndrome, and steroid-resistance (no complete remission (CR) after 8 weeks of treatment with high dose CS). We evaluated late response (CR or partial remission (PR); use of second line immunosuppressive treatment; predictors for kidney function deterioration; and long-term outcome.

Results

We included 51 patients (31 males), mean age 46 years, referred to our academic clinic between 1995 and 2014. Only 6 patients were in PR at 8 weeks. High dose CS monotherapy was continued for a median of 17 weeks (IQR 11-21), with a total duration of 56 weeks (IQR 28-83). The cumulative incidence of CR and PR were 0 and 16 patients at 16 weeks and 1 and 23 patients at 24 weeks of treatment. In total, 9 patients (18%) attained a CR and 16 patients (31%) attained a PR during initial (continued) treatment with CS. Seven patients in PR were subsequently treated with other immunosuppressive therapy (IS), resulting in CR in three.
22 patients received additional immunosuppressive therapy because of persistent nephrotic range proteinuria, seven attained a CR and 10 attained a PR. Additionally, one patient attained a spontaneous CR 2.5 years after IS therapy was discontinued.
At end of FU 12 patients were in CR, 22 in PR, 17 had a (persistent or relapsed) NS. 6 patients had developed ESRD. In 29 patients eGFR was below p5 of age-matched controls.19 patients had kidney function deterioration ≥ 3ml/min/1.73m2 per year.
Older age, FSGS-tip, and CR were associated with stable kidney function. Remarkably, PR was not a predictor for a stable kidney function. The definition of PR as recently proposed by Troost et al. (a 40% reduction in proteinuria (<1.5 g/g)) was also not associated with a stable kidney function.

Conclusion

Long-term outcome in patients with steroid resistance is not uniformly dismal. Still, kidney function deterioration ≥ 3ml/min/1.73m2 per year occurs in 37%. Better biomarkers are needed to predict long term outcome in patients with iFSGS.