259 Fibrillary Glomerulonephritis: An Insidious Disease

2021 ◽  
Vol 77 (4) ◽  
pp. 648-649
Keyword(s):  
Fibrillary Glomerulonephritis

scholarly journals Fibrilo-Tactoid Glomerulonephritis: A Possible Novel Morphological Variant

2020 ◽  
Vol 10 (3) ◽  
pp. 154-162
Author(s):  
Amaresh Vanga ◽  
Sandeep Magoon ◽  
Jolanta Kowalewska ◽  
Saad Mussarat
Keyword(s):  
Clinical Implications ◽  
Pathological Features ◽  
Hollow Core ◽  
Fibrillary Glomerulonephritis ◽  
Aged Woman ◽  
Immunotactoid Glomerulopathy ◽  
Middle Aged Woman ◽  
Nephrotic Range Proteinuria ◽  
Diagnostic Importance ◽  
Immunotactoid Glomerulonephritis

Fibrillary and immunotactoid glomerulonephritis are infrequent causes of primary nephrotic range proteinuria and are poorly understood. Recent significant developments include the discovery of DNA JB9 antigen in fibrillary glomerulonephritis. Here, we present a case of a middle-aged woman who presented with nephrotic range proteinuria, hematuria, and normal renal function. Renal biopsy revealed fibrils that were randomly arranged on electron microscopy. They were of small size and congo red negative similar to the ones found in fibrillary glomerulonephritis, but were also DNA JB 9 negative, and had a hollow core like in immunotactoid glomerulopathy. Though we try to classify these conditions into either immunotactoid glomerulonephropathy (ITGN) or fibrillary glomerulonephritis (FGN), there are scenarios such as this case where it does not fit into either and is probably an overlap or intermediate variant of these two conditions. Pathological features of these glomerulonephrites are discussed together with their clinical implications, treatment choices, and diagnostic importance.

scholarly journals Characteristics of patients with coexisting DNAJB9-associated fibrillary glomerulonephritis and IgA nephropathy

2020 ◽  
Author(s):  
Samar M Said ◽  
Alejandro Best Rocha ◽  
Anthony M Valeri ◽  
Mohamad Sandid ◽  
Anhisekh Sinha Ray ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Immunoglobulin A ◽  
Renal Survival ◽  
Hepatitis C Infection ◽  
Clinicopathologic Characteristics ◽  
Fibrillary Glomerulonephritis ◽  
Kaplan Meier ◽  
Dense Deposits ◽  
End Stage

Abstract Background Coexistence of fibrillary glomerulonephritis (FGN) and immunoglobulin A (IgA) nephropathy (IgAN) in the same kidney biopsy (FGN–IgAN) is rare, and the clinicopathologic characteristics and outcome of this dual glomerulopathy are unknown. Methods In this study, 20 patients with FGN–IgAN were studied and their characteristics were compared with 40 FGN and 40 IgAN control patients. Results Concurrent IgAN was present in 1.8% of 847 consecutive FGN cases and was the second most common concurrent glomerulopathy after diabetic nephropathy. FGN–IgAN patients were overwhelmingly White (94%) and contrary to FGN patients were predominantly (60%) males. Compared with IgAN patients, FGN–IgAN patients were older, had higher proteinuria, a higher incidence of renal insufficiency, and a lower incidence of microhematuria and gross hematuria at diagnosis. Six (30%) patients had malignancy, autoimmune disease or hepatitis C infection, but none had a secondary cause of IgAN or clinical features of Henoch–Schonlein purpura. Histologically, all cases exhibited smudgy glomerular staining for immunoglobulin G and DnaJ homolog subfamily B member 9 (DNAJB9) with corresponding fibrillary deposits and granular mesangial staining for IgA with corresponding mesangial granular electron-dense deposits. On follow-up (median 27 months), 10 of 18 (56%) FGN–IgAN patients progressed to end-stage kidney disease (ESKD), including 5 who subsequently died. Serum creatinine at diagnosis was a poor predictor of renal survival. The proportion of patients reaching ESKD or died was higher in FGN–IgAN than in IgAN. The median Kaplan–Meier ESKD-free survival time was 44 months for FGN–IgAN, which was shorter than IgAN (unable to compute, P = 0.013) and FGN (107 months, P = 0.048). Conclusions FGN–IgAN is very rare, with clinical presentation and demographics closer to FGN than IgAN. Prognosis is guarded with a median renal survival of 3.6 years. The diagnosis of this dual glomerulopathy requires careful evaluation of immunofluorescence findings, and electron microscopy or DNAJB9 immunohistochemistry.

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

2017 ◽  
Vol 45 (3) ◽  
pp. 248-256 ◽  
Author(s):  
Fernanda Payan Schober ◽  
Meghan A. Jobson ◽  
Caroline J. Poulton ◽  
Harsharan K. Singh ◽  
Volker Nickeleit ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Disease Process ◽  
Signs And Symptoms ◽  
Patient Characteristics ◽  
Underlying Disease ◽  
Fibrillary Glomerulonephritis ◽  
Black Patients ◽  
End Stage ◽  
20 Nm ◽  
The University

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 glomerulonephritis or complement 3 glomerulopathy: a rare case of diffuse necrotising crescentic glomerulonephritis with C3-dominant glomerular deposition and positive DNAJB9

2021 ◽  
Vol 14 (2) ◽  
pp. e239868
Author(s):  
Lyle Wesley Baker ◽  
Mahnoor Khan ◽  
Cherise Cortese ◽  
Nabeel Aslam
Keyword(s):  
Rare Case ◽  
Crescentic Glomerulonephritis ◽  
Complement Pathway ◽  
Hepatitis C Infection ◽  
Fibrillary Glomerulonephritis ◽  
Complement 3 ◽  
Nephritic Syndrome ◽  
Mean Diameter ◽  
Alternative Complement ◽  
Glomerular Deposits

Fibrillary glomerulonephritis (FGN) and complement 3 glomerulopathy (C3G) are rare forms of glomerulonephritis with distinct aetiologies. Both FGN and C3G can present with nephritic syndrome. FGN is associated with autoimmune disease, dysproteinaemia, malignancy and hepatitis C infection. C3G is caused by the unregulated activation of the alternative complement pathway. We present a rare case of diffuse necrotising crescentic glomerulonephritis with dominant C3 glomerular staining on immunofluorescence—consistent with C3G—but electron microscopy (EM) findings of randomly oriented fibrils with a mean diameter of 14 nm and positive immunohistochemistry for DNAJB9—suggestive of FGN. To the best of our knowledge, this is the first reported case of FGN to show dominant C3 glomerular deposits. This case report reaffirms the utility of EM in the evaluation of nephritic syndrome and highlights the value of DNAJB9—a novel biomarker with a sensitivity and specificity near 100% for FGN.

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