Abstract: FR-PO1055
Fibrillary Glomerulonephritis: Clinical Features and Outcomes in an Ethnically Diverse Urban European Population
Session Information
- Glomerular Diseases: Immunology and Inflammation - II
October 26, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Nyunt, Sandhi Wynn, University Hospitals Birmingham, Birmingham, United Kingdom
- Skordilis, Kassiani, University Hospitals Birmingham, Birmingham, United Kingdom
- Hewins, Peter, University Hospitals Birmingham, Birmingham, United Kingdom
- Berry, Miriam, University Hospitals Birmingham, Birmingham, United Kingdom
Background
Fibrillary glomerulonephritis (FGN) is a rare glomerular lesion characterised by the presence of 20nm fibrillary deposits on electron microscopy (EM). It is usually associated with poor renal outcomes. The largest case series to date describe North American populations.
Methods
We performed a retrospective case note review of patients with FGN, identified through the renal histology database at our tertiary referral hospital in the UK from 2001-17. EM has been performed routinely on all native biopsies since 2014.
Results
We identified 19 patients with FGN. 7 had a second histological lesion present including: IgA disease (2), vasculitis (4) nodular glomerulosclerosis (1). The median age at presentation was 58 (47-72) years, with a female preponderance (1.7:1 F:M). 83% were Caucasian. There were no strong associations with systemic disease. At presentation, median eGFR was 44 (20-68) ml/min and urine albumin:creatinine ratio 264 (80-351) mg/mmol. Hypertension (35%), proteinuria (88%), haematuria (71%) and hypercholesterolaemia (71%) were common while nephrotic syndrome was rare (11%).
38% patients received immunomodulatory therapy: corticosteroids (n=6), mycophenolate mofetil (n=2), azathioprine (n=1), cyclophosphamide (n=1). After median 44 (22-49) months follow-up, 47% patients were clinically stable, 6% had progressive CKD, 35% had progressed to end stage renal disease (ESRD) and 12% had died. Of those patients receiving immunomodulatory therapy, n=3 were stable, n=2 progressed to ESRD and n=1 had died. Renal insufficiency and significant proteinuria at diagnosis were risk factors for poor renal outcomes. Patients with FGN alone had significantly better renal outcomes than those with a second histological lesion; 75% of patients with vasculitic lesions progressed to ESRD.
Conclusion
Diagnosis of FGN has become more common with routine use of EM. FGN was more common in Caucasian patients, even in an ethnically diverse population. In contrast to previous studies, there were no strong associations with systemic disease. The efficacy of immunomodulatory therapy is difficult to evaluate in a small sample size. Significant proteinuria and renal insufficiency were poor prognostic indicators. Renal outcomes were better than in published case series; longer follow-up is required to determine the significance of this observation.