Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis.
BACKGROUND
Fibrillary glomerulonephritis is characterized by randomly arranged fibrils, approximately 20 nm in diameter by electron microscopy. Patients present with proteinuria, hematuria and kidney insufficiency, and about half of the reported patients progress to end-stage kidney disease within 4 years. The dependence of patient characteristics and outcomes on race has not been explored. In this study, we describe a cohort of patients with fibrillary glomerulonephritis and compare their clinical characteristics and outcomes with those of patients previously described.


METHODS
The University of North Carolina (UNC) Nephropathology Database was used to retrospectively identify patients diagnosed with fibrillary glomerulonephritis between 1985 and 2015. Of these patients, those treated at UNC were selected. Their demographic and clinical characteristics - including signs and symptoms, comorbidities, laboratory values, treatments and outcomes - were compared with those of patients described earlier.


RESULTS
Among the 287 patients identified, 42 were treated at the UNC Kidney Center. When compared to earlier cohorts, a higher frequency of black race, hepatitis C virus (HCV) infection and use of hemodialysis were noted in both black and HCV-positive patients. Autoimmune diseases, infections and malignancies were frequently observed, present in over half of all cases.


CONCLUSIONS
According to this study, fibrillary glomerulonephritis represents a secondary glomerular disease process (associated with autoimmune disease, infection or malignancy) in many cases and hence screening is essential. As the screening for comorbidities increased over time, more underlying causes were identified. We noted a high frequency of HCV among black patients, suggesting a possible causative association. Treatment of underlying disease is essential for patients for the best outcome.
University of North Carolina (UNC) Kidney Center, Division of Nephrology and Hypertension, Department of Medicine, UNC School of Medicine, Chapel Hill, NC, USA.
Fernanda
F
Payan Schober
Meghan A
MA
Jobson
Caroline J
CJ
Poulton
Harsharan K
HK
Singh
Volker
V
Nickeleit
Ronald J
RJ
Falk
J Charles
JC
Jennette
Patrick H
PH
Nachman
William F
WF
Pendergraft Iii
D001741:Black or African American; D000368:Aged; D001706:Biopsy; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000208:ethnology* / Q000628:therapy*; D006801:Humans; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000150:complications / Q000208:ethnology* / Q000628:therapy*; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009657:North Carolina; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D014495:Universities