Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis

Abstract

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. Conclusion: 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.

Fibrillary Glomerulopathy with a High Level of Myeloperoxidase-ANCA: A Case Report

“…However, because diseases other than AAV are sometimes positive for ANCA [13], the interpretation of its staining is difficult except in typical vasculitis. In addition, systemic diseases such as autoimmune diseases, malignant tumors, and chronic infections are known to be associated with 1/3 rd of FGN cases [14]. e pathogenicity of ANCA is thought to be due to differences in its epitopes [15]; however, there is no ideal way to distinguish between them.…”

Rituximab and Fibrillary Glomerulonephritis: Interest of B Cell Reconstitution Monitoring

“…Rituximab therapy has recently emerged as a promising alternative therapeutic option to halt the progression of renal disease [ 8 , 9 , 10 ]. A recent study from the University of Northern Carolina showed that of nine rituximab-treated patients, only 11% achieved a stable condition, however those treated earlier and those who had less sclerosis at the time of biopsy responded better to rituximab treatment [ 11 ]. Due to missing data concerning optimal dose of rituximab to treat FGN, our initial protocol (two doses of 375 mg/m 2 separated by one week) was largely extrapolated from the use of rituximab in other glomerular diseases [ 12 , 13 ].…”

Fibrillary Glomerulonephritis and DnaJ Homolog Subfamily B Member 9 (DNAJB9)

“…Solid and hematologic malignancies are common in patients with FGN. 10,13 The prevalence of malignancy in these cohorts have ranged between 4 to 23%. 10,11,13 No causal relationship has been established thus far between malignancy and FGN.…”

“…10,13 The prevalence of malignancy in these cohorts have ranged between 4 to 23%. 10,11,13 No causal relationship has been established thus far between malignancy and FGN. It is possible that there are coexisting risk factors (such as similar age groups) that increase both the risk of malignancy and FGN.…”

“…Hepatitis C virus (HCV) is the most common viral infection associated with FGN (ranging between 3 to 27%) and more commonly seen in African Americans. 10,11,13 Hepatitis B, on the other hand, is much less common. 13 Human immunodeficiency virus (HIV) infection is also linked to FGN but most cases are with concurrent HCV infection.…”