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Abstract: SA-PO655

Pauci-Immune Glomerulonephritis (PIGN) with Low Clearance at Clinical Presentation: Predictors of Treatment Response and Long-Term Outcomes

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Lionaki, Sophia, Laiko Hospital , Athens, Greece
  • Marinaki, Smaragdi, Laiko Hospital , Athens, Greece
  • Kalogeropoulos, Petros, Laiko Hospital , Athens, Greece
  • Liapis, Georgios, Laiko Hospital , Athens, Greece
  • Kapsia, Helen, Laiko Hospital , Athens, Greece
  • Gakiopoulou, Harikleia, Laiko Hospital , Athens, Greece
  • Tzioufas, Athanasios, Laiko Hospital , Athens, Greece
  • Boletis, John N., Laiko Hospital , Athens, Greece
Background

Pauci-immune vasculitis often presents with severe kidney involvement requiring hemodialysis. Considering the poor prognosis in this setting, we aimed to explore the factors which are associated with response to therapy.

Methods

Patients were included if they had biopsy proven PIGN with estimated GFR<20 ml/min/1.73 m2or required dialysis at presentation, received standard immunosuppression and had a follow up > 6 months. We recorded clinical, laboratory and histopathological parameters at diagnosis, at 3 months, at 1 year and at the end of follow up. Outcomes of interest included response to treatment, ESKD, and death. Treatment response was defined by the ability to come off dialysis with an eGFR>20 ml/min/1.73 m2 with no signs of vasculitis. Histopathological evaluation included arteriosclerosis, % of normal glomeruli, activity index, chronicity index.

Results

A total of 77 patients, with a mean age of 60.6(16.05) years were included. There were, 42 males (54.5%). After 3 months, 55 patients (74.6%) had responded to immunosuppressive therapy, 15 (20%) were dialysis depended, 5(6.7%) died and 2 were lost in follow up. By the end of the 1st year, 54 patients (71.4%) achieved remission, 15(20%) ended up in ESKD and 6(8%) died. Factors which were associated with treatment response included MPO-ANCA positivity [odds ratio OR:3.9, 95%CI(1.13-13.37) p=0.03], eGFR>10ml/min/1.73m2 at presentation [OR:2.5, 95% CI(0.86-7.30), p=0.009], normal glomeruli >10% [OR:3.8, 95%CI(1.24-12.1), p=0.02], and chronicity index more than 6 [OR:6.2, 95% CI(1.77-22.4),p=0.004]. Risk factors associated with ESKD included non-response to immunosuppressive therapy [Relative Risk RR:0.05, 95%CI(0.01-0.2) p<0.0001)], normal glomeruli<10% in the diagnostic biopsy [RR: 2.9, 95% CI(1.38-6.32), p=0.005] and age>75 years [RR:3.2, 95% CI(0.9-10.6) p=0.055]. Two of the 6 deaths were disease related.

Conclusion

A significant proportion of patients with PIGN who presented with severe renal dysfunction, responded to immunosuppresive therapy and recovered renal function approximately 3 months after initiation of therapy. Risk factors for ESKD were age>75 years, <10% normal glomeruli in the diagnostic biopsy and non-response to immunosuppressive therapy.