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"Fibrillary glomerulonephritis"

H H Shah, J Thakkar, J M Pullman, A T Mathew
Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that commonly presents clinically with hypertension, proteinuria, microscopic hematuria, and varying degree of renal insufficiency. Histologically, FGN can present with different patterns of glomerular injury, more commonly mesangioproliferative, membranoproliferative, and membranous nephropathy. While crescent formation has been described in some kidney biopsy series of FGN, crescentic glomerulonephritis pattern of glomerular injury has been rarely described...
March 2017: Indian Journal of Nephrology
Fernanda Payan Schober, Meghan A Jobson, Caroline J Poulton, Harsharan K Singh, Volker Nickeleit, Ronald J Falk, J Charles Jennette, Patrick H Nachman, William F Pendergraft Iii
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...
2017: American Journal of Nephrology
Gabrielle Normand, Anne Jolivot, Maud Rabeyrin, Valérie Hervieu, Pierre-Jean Valette, Jean-Yves Scoazec, Jean-Michel Gougon, Laurent Juillard, Jérôme Dumortier
A 50-year-old man presented with nephrotic syndrome. Electron microscopy analysis of a kidney biopsy specimen showed fibrillary glomerulonephritis, a rare glomerular disease, while histological analysis of a liver tumor biopsy confirmed an intrahepatic cholangiocarcinoma. The paraneoplastic nature of fibrillary glomerulonephritis is debated but after curative treatment of the hepatic nodule, remission of nephrotic syndrome was confirmed at 6-, 12- and 24-months follow-up. To our knowledge, this is the first description of a paraneoplastic fibrillary glomerulonephritis associated with a cholangiocarcinoma, supported by complete remission achieved following cancer treatment...
August 16, 2016: Clinics and Research in Hepatology and Gastroenterology
Arvind Madan, Snezana Mijovic-Das, Ana Stankovic, Geoffrey Teehan, Amber S Milward, Anupa Khastgir
BACKGROUND: Current first-line anti-proteinuric treatments for nephrotic syndrome (NS) do not produce an effective response in all patients and are not tolerated by some patients. Additional effective and tolerable treatment options in NS are strongly needed. This retrospective case series is the largest to date to examine Acthar gel (adrenocorticotropic hormone, ACTH) in patients with varied-etiology NS. METHODS: This multicenter retrospective case series included adult patients with NS (N = 44) treated with Acthar gel at 6 clinical practices...
March 31, 2016: BMC Nephrology
Zhao-Yu Lu, Hai-Feng Yang, Yu Peng, Yin Li, Zi-Chang Yin, Fu-Hua Lu, Xu-Sheng Liu
No abstract text is available yet for this article.
May 2016: Chinese Journal of Integrative Medicine
Brian T Brinkerhoff, Donald C Houghton, Megan L Troxell
Renal injury in hematopoietic cell transplant recipients may be related to a combination of factors including chemotherapy, radiation, infection, immunosuppressive agents, ischemia, and graft-versus-host disease, and can involve glomerular, tubulointerstitial, and vascular structures. We reviewed renal pathology from 67 patients at a single institution (2009-2014), including 14 patients with biopsy for clinical dysfunction, 6 patients with surgical kidney resection for other causes, and 47 autopsy patients...
June 2016: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Chung-Kuan Wu, Jyh-Gang Leu, An-Hang Yang, Der-Cherng Tarng, Hsiang-Yuen Tung, Shou-Shan Chiang
BACKGROUND: Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease that seldom coexists with other diseases. Membranoproliferative glomerulonephritis is a pathologic finding of renal lesions associated with IgM-secreting monoclonal proliferations. We present a case study of a patient with unusual simultaneous FGN and IgM-related renal disorder in nonmalignant monoclonal IgM gammopathy. CASE PRESENTATION: A 63-year-old male presented with nephrotic syndrome and elevated serum creatinine levels...
February 18, 2016: BMC Nephrology
Jessica Hemminger, Gyongyi Nadasdy, Anjali Satoskar, Sergey V Brodsky, Tibor Nadasdy
Immunofluorescence staining plays a vital role in nephropathology, but the panel of antibodies used has not changed for decades. Further classification of immunoglobulin (Ig)G-containing immune-type deposits with IgG subclass staining (IgG1, IgG2, IgG3, and IgG4) has been shown to be of diagnostic utility in glomerular diseases, but their value in the evaluation of renal biopsies has not been addressed systematically in large renal biopsy material. Between January 2007 and June 2014, using direct immunofluorescence, we stained every renal biopsy for the IgG subclasses if there was moderate to prominent glomerular IgG staining and/or IgG-predominant or IgG-codominant glomerular staining...
May 2016: American Journal of Surgical Pathology
Guy Touchard, Frank Bridoux, Jean-Michel Goujon
The spectrum of glomerular disorders with organized immunoglobulin (Ig) deposits is heterogeneous. It encompasses 2 mains categories: glomerulopathies with fibrillary deposits are mostly represented by immunoglobulinic amyloidosis (most commonly AL amyloidosis, characterized by monoclonal light chain deposits often of the lambda isotype), and pseudo-amyloid fibrillary glomerulonephritis in which deposits predominantly contain polyclonal IgG4. Glomerulopathies with microtubular deposits include cryoglobulinemic glomerulonephritis (type I and type II, with or without detectable serum cryoglobulin) and glomerulonephritis with organized microtubular monoclonal Ig deposits (GOMMID) also referred to as immunotactoid glomerulopathy...
February 2016: Néphrologie & Thérapeutique
Wisit Cheungpasitporn, Claudia C Zacharek, Fernando C Fervenza, Lynn D Cornell, Sanjeev Sethi, Loren P Herrera Hernandez, Samih H Nasr, Mariam P Alexander
Anti-glomerular basement membrane (anti-GBM) disease is a major cause of rapidly progressive glomerulonephritis (RPGN). On the other hand, fibrillary glomerulonephritis (GN) typically presents as proteinuria, hematuria and renal insufficiency, but rarely as RPGN. Without electron microscopy, the diagnosis of fibrillary GN can be missed. We report a 68-year-old white woman who presented with RPGN with kidney biopsy demonstrating diffuse crescentic GN on light microscopy. By immunofluorescence, there was bright linear staining of the GBMs and smudgy mesangial staining for immunoglobulin G, C3, and kappa and lambda light chain...
February 2016: Clinical Kidney Journal
Georges N Nakhoul, James F Simon
Fibrillary glomerulonephritis (GN) is a rare glomerular disorder that has been associated with monoclonal gammopathies, malignancies, chronic infections, and autoimmune disorders. We present the case of a 56-year-old woman with limited-type scleroderma and remote discoid lupus, evaluated for dipstick positive hematuria and preserved kidney function. Serologies were negative. Kidney biopsy revealed fibrillary GN. Her renal function and proteinuria remain stable 4 years after her initial diagnosis. This case is unusual both in its presentation and evolution, but mostly because it is the first reported case of fibrillary GN in association with limited type scleroderma...
April 2016: Clinical Nephrology
Jimmy A Thomas, Dmitri Vasin, Mercury Lin, Arthur E Anderson, Charles E Alpers
Fibrillary glomerulonephritis (FGN) is a rare cause of rapidly progressive glomerulonephritis (RPGN). We report a case of FGN in which the patient presented with a clinical pulmonary-renal syndrome and whose kidney biopsy showed > 90% crescents on light microscopy. Immunofluorescence microscopy showed pseudo-linear IgG and C3 staining of the glomerular capillary walls resulting in an initial diagnosis of crescentic glomerulonephritis of anti-glomerular basement membrane (anti-GBM) antibody etiology. Electron microscopy showed fibrillary deposits permeating the glomerular capillary walls, characteristic of FGN...
February 2016: Clinical Nephrology
Woo Kyung Sung, Jin Uk Jeong, Ki Tae Bang, Jong Ho Shin, Ji Hyung Yoo, Nak Min Kim, Jun Hyung Park, Joo Heon Kim
A 58-yr-old man presented with leg edema and subacute weakness of his bilateral lower extremities. Urinary and serum immunoelectrophoresis revealed the presence of lambda-type Bence Jones proteins. He was ultimately diagnosed with monoclonal gammopathy of undetermined significance (MGUS). A renal biopsy specimen showed fibrillary glomerulonephritis (FGN), which was randomly arranged as 12-20 m nonbranching fibrils in the basement membranes. Immunofluorescence studies were negative for immunoglobulin (Ig)G, IgM, IgA, C3, and kappa light chains in the capillary walls and mesangial areas...
June 2015: Kidney Research and Clinical Practice
Andrew Mallett, Wen Tang, Gareth Hart, Stephen P McDonald, Carmel M Hawley, Sunil V Badve, Neil Boudville, Fiona G Brown, Scott B Campbell, Philip A Clayton, David W Johnson
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...
2015: American Journal of Nephrology
Mark A Lusco, Agnes B Fogo, Behzad Najafian, Charles E Alpers
No abstract text is available yet for this article.
October 2015: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Ikuyo Narita, Michiko Shimada, Takeshi Fujita, Reiichi Murakami, Masayuki Nakamura, Norio Nakamura, Hideaki Yamabe, Ken Okumura
BACKGROUND: It is sometimes challenging to diagnose unsusual cases of fibrillary glomerulonephritis (FGN) and immunotactoid glomerulopathy (ITG), the rare causes of nephrotic syndrome. CASE PRESENTATION: A 75-year-old Japanese woman presented with nephrotic syndrome, microhematuria and renal insufficiency. Renal biopsy revealed membranoproliferative glomerulonephritis (MPGN) with IgM and weak C3 deposition. Congo red stain was negative. Electron microscopy demonstrated massive fibrils in the subendothelium, mesangium and subepithelium...
2015: BMC Nephrology
Amr El-Husseini, Juan-Carlos Aycinena, Bennet George, Stuart Jennings, Virgilius Cornea, B Peter Sawaya
Fibrillary glomerulonephritis (FGN) is a rare disorder with poor renal prognosis. It is a heterogeneous disease associated with significant risk of end-stage renal disease (ESRD). Its etiology and pathogenesis have not been clearly identified. We report a case of a patient presenting with hypertensive crisis, nephrotic range proteinuria, and rapidly progressive glomerulonephritis (RPGN). The kidney biopsy demonstrates crescentic GN on light microscopy (LM) and strong pseudo-linear/globular glomerular basement membrane (GBM) staining for immunoglobulin G on immunofluorescence (IF), suggestive of anti-GBM disease...
October 2015: Clinical Nephrology
Valeria Sainz-Prestel, Jesus Hernandez-Perez, Jorge Rojas-Rivera, José María Milicua-Muñoz, Jesus Egido, Alberto Ortiz
Rituximab (RTX) is a chimeric monoclonal antibody against CD20+ B cells increasingly used to treat kidney disorders. RTX-induced pulmonary disease has been reported in patients treated for haematological disorders, and a few cases have been observed in patients with underlying rheumatological conditions. We report a case of non-infectious interstitial pneumonitis associated with RTX use in a 49-year-old patient with primary (fibrillary) glomerulonephritis. As typically observed, discontinuation of the drug and prompt initiation of glucocorticoids led to resolution of pulmonary manifestations...
October 2013: Clinical Kidney Journal
Tracey Ying, Prue Hill, Michael Desmond, John Agar, Andrew Mallett
Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non-amyloid fibrillary deposits of unknown aetiology. It is generally considered idiopathic but may be associated with secondary causes such as monoclonal gammopathy, hepatitis B and C infections, autoimmune diseases and malignancies. We report two Australian families with apparent familial fibrillary glomerulonephritis inherited in an autosomal dominant pattern, and postulate the existence of a primary familial entity. Family 1 consists of an affected father and daughter; the daughter progressed to end-stage renal failure within 18 months of diagnosis, despite immunosuppressive therapy...
July 2015: Nephrology
Shalini Nilajgi, John Paul Killen, Richard Baer, Patricia Renaut, Murty Mantha
We report an unusual case of fibrillary glomerulonephritis (FGN) presenting as rapidly progressive renal failure and extensive crescent formation along with linear staining of capillary walls of the glomeruli on immunofluorescence, mimicking anti-glomerular basement membrane (anti-GBM) antibody-mediated disease. Laboratory results for circulating anti-GBM antibodies were negative. The subsequent electron microscopic findings were that of presence of electron-dense deposits in the glomerular mesangium and capillary walls, comprising of non-branching fibrils with an average diameter of 16 nm consistent with a diagnosis of FGN...
December 2011: NDT Plus
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