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Abstract: PO1859

Fibrillary Glomerulonephritis: A Case Series with Clinical Features and Outcomes

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Zonozi, Reza, Massachusetts General Hospital, Boston, Massachusetts, United States
  • Laliberte, Karen A., Massachusetts General Hospital, Boston, Massachusetts, United States
  • Rosenthal, Jillian, Massachusetts General Hospital, Boston, Massachusetts, United States
  • Huizenga, Noah, Massachusetts General Hospital, Boston, Massachusetts, United States
  • Dowst, Sarah, Massachusetts General Hospital, Boston, Massachusetts, United States
  • Jeyabalan, Anushya, Massachusetts General Hospital, Boston, Massachusetts, United States
  • Cortazar, Frank B., New York Nephrology Vasculitis and Glomerular Center, Albany, New York, United States
  • Niles, John, Massachusetts General Hospital, Boston, Massachusetts, United States
Background

Fibrillary glomerulonephritis (FGN) is a rare cause of chronic glomerulonephritis with a poor prognosis. We evaluated a series of patients with FGN, most of whom were treated with rituximab, low-dose cyclophsophamide, and prednisone (RCP).

Methods

Patients were included if they had FGN treated at Massachusetts General Hospital between 2008 – 2020 with a minimum of six months of follow up. The primary outcome was achievement of remission, defined as serum creatinine that remained stable, improved or increased <25% of the original value after treatment, and a 50% reduction in proteinuria at the end of follow up.

Results

We identified 11 consecutive patients with FGN. ANCA-associated vasculitis (n = 3), rheumatoid arthritis (n = 1), chronic hepatitis C (n = 1), and monoclonal B lymphocytosis (n = 1) were concurrently present. At the start of therapy, the median (IQR) serum creatinine, eGFR, and protienuria was 2.31 mg/dL (1.25 – 4.69), 30 mL/min/1.73 m2 (11 – 68), and 6.6 g/g (1.5 – 8.4), respectively. Of the 11 patients, 10 were treated with RCP, and one patient with rituximab monotherapy. The median (IQR) follow-up was 2.6 years (2.0 – 3.9). Seven of 11 patients achieved remission. Of the 4 patients who did not achieve remission, one received pre-emptive transplantation, two initiated hemodialysis, and one had > 25% rise in serum creatinine not reaching ESRD. Five serious adverse events occurred over 33 patient years.

Conclusion

Remission was achieved in most patients with FGN treated with rituximab, low-dose cyclophosphamide, and prednisone. Larger studies evaluating this regimen are warranted.