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Abstract: TH-PO651

The Clinico-Pathologic Characteristics of Patients with Fibrillary Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Osman, Omar, Cleveland Clinic, Cleveland, Ohio, United States
  • Sawaf, Hanny, Cleveland Clinic, Cleveland, Ohio, United States
  • Gudura, Tariku T., Cleveland Clinic, Cleveland, Ohio, United States
  • Herlitz, Leal C., Cleveland Clinic, Cleveland, Ohio, United States
  • Mehdi, Ali, Cleveland Clinic, Cleveland, Ohio, United States
  • Gebreselassie, Surafel K., Cleveland Clinic Florida, Weston, Florida, United States
  • Bobart, Shane A., Cleveland Clinic Florida, Weston, Florida, United States
Background

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with lack of sufficient understanding of the disease pathogenesis. As a result, there is a lack of good evidence to guide therapy.

Methods

We identified patients with FGN from the Cleveland Clinic Kidney Biopsy Epidemiology Project from January 2015 to March 2023. Retrospective chart review was performed to obtain demographic and clinical characteristics.

Results

From 45 patients with FGN, 30 patients had sufficient data. 70% were female and 86% self-reported as white. The median age was 64 years. Clinical features at kidney biopsy were: hypertension (100%), acute or chronic kidney disease (100%), hematuria (76%), proteinuria (60%), nephrotic syndrome (48%). Hypocomplementemia was identified in one case. Work up for secondary associations revealed, M protein (20%), Hepatitis C virus infection (16%), autoimmune conditions (23%) and solid tumor malignancy (10%). Mean serum creatinine and proteinuria at biopsy were 2.7mg/dl (0.7-10) and 3.6 gm/dl (0.1-8.7) respectively.
Regarding pathology, pure mesangial (90%) followed by endocapillary hypercellularity (6.7%) were the most common patterns of injury. The majority of patients (76%) had moderate to severe interstitial fibrosis and tubular atrophy and 13% had crescents. DNAJB9 was tested on 2 samples, both were positive. 86% of patients had positive IF staining for IgG/C3/K/L with the majority of IgG staining being described as a smudgy pattern. EM showed mesangial pattern deposition and GBM thickening in more than 80% of patients.
Regarding treatment and outcomes, most patients were treated with non-immuno-modulatory antiproteinuric agents. Of 7 patients treated with immunosuppressive therapy, 5 patients received Rituximab. During an average of 24 months (0-70) follow up for 13 patients with data, 23% had partial remission (proteinuria reduction > 50% with stable eGFR), 23% had persistent disease, and 53% (7) progressed to ESKD. Aside from a positive correlation between creatinine during biopsy and ESRD, no predictive variables were identified on univariate and multivariable analysis.

Conclusion

FGN is a rare glomerular disease with poor renal outcomes. Treatment strategies vary. Prospective, multicenter studies are necessary to better understand the disease pathogenesis and identify appropriate therapy.

Funding

  • Private Foundation Support