scholarly journals Fibrillary Glomerulonephritis and Immunotactoid Glomerulopathy

2007 ◽  
Vol 19 (1) ◽  
pp. 34-37 ◽  
Author(s):  
Charles E. Alpers ◽  
Jolanta Kowalewska
Keyword(s):  
Fibrillary Glomerulonephritis ◽  
Immunotactoid Glomerulopathy

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 Hepatitis C viral infection is associated with fibrillary glomerulonephritis and immunotactoid glomerulopathy.

1998 ◽  
Vol 9 (12) ◽  
pp. 2244-2252
Author(s):  
G S Markowitz ◽  
J T Cheng ◽  
R B Colvin ◽  
W M Trebbin ◽  
V D D'Agati
Keyword(s):  
Hepatitis C ◽  
Glomerular Disease ◽  
Hcv Infection ◽  
Not Given ◽  
Fibrillary Glomerulonephritis ◽  
Persistent Proteinuria ◽  
Immunotactoid Glomerulopathy ◽  
Membranous Glomerulopathy ◽  
Cryoglobulinemic Glomerulonephritis

The most common form of glomerular disease seen in association with hepatitis C virus (HCV) infection is membranoproliferative glomerulonephritis, with or without associated cryoglobulinemia. This study examines four cases of fibrillary glomerulonephritis and two cases of immunotactoid glomerulopathy in association with HCV infection. Findings at presentation included proteinuria, renal insufficiency, and hematuria. Renal biopsy revealed a membranoproliferative pattern of glomerular disease in five cases, and a membranous glomerulopathy with mesangial proliferative features in one. On immunofluorescence, all cases stained with IgG and C3. Electron microscopy revealed fibrils of the expected diameter, 16 to 28 nm in fibrillary glomerulonephritis and 33 to 45 nm in immunotactoid glomerulopathy. In only one case were cryoglobulins detected (at low titer and on only one of three assays). Antiviral therapy was not given in any of the six cases. Outcomes were mixed, with progression to renal failure occurring in two patients and persistent proteinuria with stable or improved renal function in three. Follow-up is not available on the sixth case. Both fibrillary glomerulonephritis and immunotactoid glomerulopathy have features that overlap with cryoglobulinemic glomerulonephritis. The relatedness of these three entities in a subset of patients with HCV infection suggests a common pathogenic mechanism of glomerular deposition of organized deposits.

scholarly journals Glomerulonephritis with associated hypocomplementemia and crescents: an unusual case of fibrillary glomerulonephritis.

1995 ◽  
Vol 6 (2) ◽  
pp. 171-176
Author(s):  
D B Adey ◽  
B R MacPherson ◽  
G C Groggel
Keyword(s):  
Unusual Case ◽  
Fibrillary Glomerulonephritis ◽  
Pathologic Features ◽  
Immunotactoid Glomerulopathy

Fibrillary glomerulonephritis is an unusual, but not rare cause of glomerulonephritis. Hypocomplementemia in association with fibrillary glomerulonephritis has been reported only once previously. A patient with hypocomplementemia and fibrillary deposits as demonstrated by electronmicroscopy is reported. The clinical and pathologic features of fibrillary glomerulonephritis and immunotactoid glomerulopathy are reviewed.

End-Stage Kidney Disease Due to Fibrillary Glomerulonephritis and Immunotactoid Glomerulopathy - Outcomes in 66 Consecutive ANZDATA Registry Cases

2015 ◽  
Vol 42 (3) ◽  
pp. 177-184 ◽  
Author(s):  
Andrew Mallett ◽  
Wen Tang ◽  
Gareth Hart ◽  
Stephen P. McDonald ◽  
Carmel M. Hawley ◽  
...  
Keyword(s):  
Renal Transplant ◽  
Kidney Disease ◽  
Renal Allograft ◽  
Patient Survival ◽  
Allograft Survival ◽  
End Stage Kidney Disease ◽  
Fibrillary Glomerulonephritis ◽  
Renal Allografts ◽  
Immunotactoid Glomerulopathy ◽  
End Stage

Background: Fibrillary glomerulonephritis (FGN) and immunotactoid glomerulopathy (IG) are uncommon and characterised by non-amyloid fibrillary glomerular deposits. The aim of this study was to investigate characteristics and outcomes of patients undergoing renal replacement therapy (RRT) for end-stage kidney disease (ESKD) secondary to FGN and IG. Methods: All ESKD patients who commenced RRT in Australia and New Zealand 1 January 1990 to 31 December 2010 were included. Outcomes were assessed by Kaplan-Meier, multivariate logistic-regression analysis and multivariable Cox proportional-hazards survival analysis. Results: Of 45,216 individuals with ESKD, 55 (0.12%) had FGN and 11 (0.02%) had IG. The median survival of FGN patients on dialysis (5.63 years, 95% CI 3.31-7.96) was not significantly different from patients with other ESKD causes (median 4.01 years, 95% CI 4.34-4.47; log-rank 1.32, p = 0.25), but was significantly longer than that of IG patients (median 2.93 years, 95% CI 0.00-6.17; log-rank 4.8, p = 0.03). Thirteen (24%) FGN patients received 13 renal-allografts, 4 (36%) IG patients received 4 renal-allografts and 11,528 (26%) other ESKD patients received 12,278 renal-allografts. FGN patients experienced comparable outcomes to other ESKD patients for both 10-year patient survival (100 vs. 84%, p = 0.93) and renal-allograft survival (67 vs. 76%, p = 0.06). For IG, the median follow-up was 3.66 years with 75% patient survival and 100% renal-allograft survival. One (8%) FGN patient and 1 (25%) IG patient experienced recurrent FGN and IG respectively in their allograft. Conclusion: Patients with FGN have comparable dialysis and renal transplant outcomes to patients with other causes of ESKD. IG patients have inferior survival on dialysis, although renal transplant outcomes are acceptable. Disease recurrence in renal-allografts was low for both FGN and IG.

Other primary glomerular diseases

2009 ◽  
pp. 435-470 ◽  
Author(s):  
Claudio Ponticelli ◽  
Richard Glassock
Keyword(s):  
Electron Microscopy ◽  
Rare Entity ◽  
Mesangial Matrix ◽  
Glomerular Diseases ◽  
Fibrillary Glomerulonephritis ◽  
Current Opinion ◽  
Primary Condition ◽  
Immunotactoid Glomerulopathy ◽  
Primary Glomerular Disease ◽  
Reduced Renal Function

Fibrillary glomerulonephritis (FGN) and immunotactoid glomerulopathy (ITG) are both glomerular diseases characterized histologically by a diffuse increase in mesangial matrix due to deposits having a fibrillary structure by electron microscopy, which are negative for Congo-red stains but positive for immunoglobulin deposition. Most patients present with proteinuria, often in a nephrotic range, microhematuria, and hypertension. Almost half of patients have a reduced renal function at presentation. Although FGN and ITG have quite similar light microscopical and clinical features the current opinion is that they represent two separate diseases. FGN is an idiopathic (primary) condition characterized by polyclonal immune deposits with restricted gamma isotypes. Most patients present with significant renal insufficiency and have a poor outcome despite immunosuppressive therapy. By contrast, ITG very often contains monoclonal IgG deposits and has a significant association with underlying monoclonal paraproteinemia and hypocomplementemia. ITG is more properly classified as a mono-clonal immunoglobulin deposition disease (MIDD). Differentiation of FGN from the much more rare entity ITG appears justified on immunopathologic, ultrastructural, and clinical grounds (Fogo et al., 1993; Bridoux et al., 2002; Rosenstock et al., 2003). Both will be discussed together, although only FGN should be regarded as a primary glomerular disease, as defined in this monograph.

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 A case of immunotactoid glomerulopathy in a patient with monoclonal gammopathy of renal significance

2021 ◽  
Vol 11 (1) ◽  
pp. 60-64
Author(s):  
Victoria Campdesuner ◽  
Yeshanew Teklie ◽  
Natalia Lattanzio ◽  
Christian Lorenzo ◽  
Stephen Bell ◽  
...  
Keyword(s):  
Monoclonal Gammopathy ◽  
Immunotactoid Glomerulopathy
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