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Membraneous Glomerulonephritis

Calvin Tsui, Pouneh Dokouhaki, Bhanu Prasad
We present a 77-year-old Caucasian woman who presented with nephrotic-range proteinuria, microhematuria, renal impairment, and extremely elevated blood pressure. She had a long history of well-controlled type 2 diabetes. Renal biopsy revealed fibrillary deposits in the mesangium and glomerular basement membrane consistent with fibrillary glomerulopathy (FGN), with crescentic changes and thrombotic microangiopathy (TMA). We could not identify any radiological, clinical, or laboratory evidence of autoimmune disorders, lymphoproliferative disorders, and malignancy...
May 2018: Case Reports in Nephrology and Dialysis
Alaa A Ali, Dana A Sharif, Safa E Almukhtar, Kais Hasan Abd, Zana Sidiq M Saleem, Michael D Hughson
BACKGROUND: Estimates of the incidence of glomerulonephritis (GN) and end-stage renal disease (ESRD) in an Iraqi population are compared with the United States (US) and Jordan. METHODS: The study set consist of renal biopsies performed in 2012 and 2013 in the Kurdish provinces of Northern Iraq. The age specific and age standardized incidence of GN was calculated from the 2011 population. ESRD incidence was estimated from Sulaimaniyah dialysis center records of patient's inititating hemodialysis in 2017...
October 11, 2018: BMC Nephrology
Kazuko Uto, Shigehisa Yanagi, Hironobu Tsubouchi, Nobuhiro Matsumoto, Masamitsu Nakazato
The concurrence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) disease, known as double-positive disease, is rare, but it occurs at a much higher frequency than expected by chance. Double-positive disease has an aggressive clinical course, with no optimal treatment strategy. Here we describe a patient with steroid-refractory double-positive disease who was treated successfully with the addition of plasma exchange (PE) and cyclophosphamide (CPA)...
2018: Respiratory Medicine Case Reports
Jayesh Sardhara, Mukesh Shukla, Janmejay Jamdar, Awadhesh Kumar Jaiswal, Sushila Jaiswal, Anupma Kaul, Kamlesh Singh Bhaisora, Kuntal Kanti Das, Anant Mehrotra, Sanjay Behari
A 60-year-old homemaker presenting with pedal edema and ascites was found to have a planum sphenoidale meningioma concurrently with nephrotic syndrome. On renal biopsy, the patient was found to have membranous glomerulonephritis. There was complete remission of nephropathy after excision of the meningioma. Nephrotic syndrome has been commonly found in association with malignancies and blood disorders but the association with a meningioma is extremely rare, and only one case has been previously reported as per our knowledge...
July 2018: Asian Journal of Neurosurgery
Rachel E Cianciolo, J Charles Jennette
Glomerulonephritis (GN) is inflammation of glomeruli. The four major categories that cause human GN are mediated by immunoglobulin or complement or both, and they include (1) immune complex-mediated GN, (2) anti-glomerular basement membrane-mediated GN, (3) antineutrophil cytoplasmic autoantibody-mediated GN, and (4) complement factor 3 glomerulopathy mediated by complement dysregulation. Initiating processes include infection, autoimmunity, exogenous antigens, and neoplasia. Often there are predisposing and modulating genetic, epigenetic, and/or environmental factors...
October 3, 2018: Toxicologic Pathology
Zhenlu Chong, Chunjing Bao, Jia He, Tianxiao Chen, Lijia Zhong, Gaopeng Li, Huanle Li, Lutong Fang, Yinjing Song, Guoxiang Fu, Xuyan Yang, Lihua Lai, Yang Liu, Qingqing Wang
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, and the pathogenesis of SLE has not been fully elucidated. The E3 ubiquitin ligase FBXW7 has been well characterized in cancer as a tumor suppressor that can promote the ubiquitination and subsequent degradation of various oncoproteins; however, the potential role of FBXW7 in autoimmune diseases is unclear. In the present study, we identified that FBXW7 is a crucial exacerbating factor for SLE development and progression in a mouse model induced by 2, 6, 10, 14-tetramethylpentadecane (TMPD)...
October 1, 2018: Cellular & Molecular Immunology
Joshua D Long, Stephanie M Rutledge, Meghan E Sise
Autoimmune kidney diseases triggered by viruses are an important cause of kidney disease in patients affected by chronic viral infection. Hepatitis B virus (HBV) infection is associated with membranous nephropathy and polyarteritis nodosa. Hepatitis C virus (HCV) infection is a major cause of cryoglobulinemic glomerulonephritis. Patients with human immunodeficiency virus (HIV) may develop HIV-associated nephropathy, a form of collapsing focal segmental glomerulosclerosis, or various forms of immune-complex-mediated kidney diseases...
November 2018: Rheumatic Diseases Clinics of North America
Kavita Gulati, Stephen P McAdoo
Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune small vessel vasculitis characterized by autoreactivity to antigens in type IV collagen chains expressed in glomerular and alveolar basement membrane. The detection of circulating anti-GBM antibodies, which are shown to be directly pathogenic, is central to disease diagnosis. Clinically, anti-GBM disease usually presents with rapidly progressive glomerulonephritis with or without alveolar hemorrhage. Rapid diagnosis and early treatment are required to prevent mortality and to preserve renal function...
November 2018: Rheumatic Diseases Clinics of North America
Johan Noble, Thomas Jouve, Paolo Malvezzi, Lionel Rostaing
Hepatitis B virus (HBV) and hepatitis C virus (HCV) infections are the major causes of chronic liver disease. HBV and HCV affect nearly 7% of the world's population. Extra-hepatic complications and particularly renal failure have different mechanisms and manifestations. The underlying mechanism, although differing for each disease, mainly involves the immune system and antibody deposits in the kidney, which can lead to tissue damage. Areas covered: We do not cover in this review hepatorenal syndrome. We report on the renal complications of viral hepatitis (HBV, HCV, hepatitis E), autoimmune hepatitis, cirrhosis, and Wilson's disease...
October 1, 2018: Expert Review of Gastroenterology & Hepatology
J Floege, P Boor, M J Moeller
Glomerulonephritides essentially all belong to the rare diseases; however, they are the most common cause of end-stage renal disease in young adults. Besides obtaining a specific diagnosis via a renal biopsy, assessing the prognosis constitutes the other essential step in the work-up, since this enables a decision to be made on whether supportive care with relatively few adverse effects is sufficient or whether additional immunosuppressive therapy is required. The latter is discussed focusing on the most common European types of glomerulonephritis: immunoglobulin A nephropathy, membranous glomerulonephritis, minimal change nephropathy and focal segmental glomerulosclerosis...
September 24, 2018: Der Internist
Dóra Bajcsi, Kypros Constantinou, László Krenács, Zsolt Barabás, Szabolcs Molnár, Szabolcs Nyiraty, György Ábrahám, Béla Iványi
Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits is characterized by granular deposits of monoclonal IgG; histologically it has typically a membranoproliferative or endocapillary pattern, and seen electronmicroscopically there are dense deposits without substructure. Here, we present the case of a 62-year-old Caucasian woman who was admitted with rapidly progressive kidney failure. The patient's status, the laboratory and imaging examinations did not support prerenal, postrenal and - among the intrinsic causes - vascular and tubulointerstitial origin...
September 2018: Orvosi Hetilap
A Carosso, M Zonca, L Colla, F Borella, L Daniele, C Benedetto
Immunoglobulin A nephropathy (IgAN) is the most common form of primary glomerulonephritis and is increasingly encountered in pregnancy. The obstetric and renal outcomes of pregnancy are controversial, however. Women with IgAN are at higher risk of hypertension, preeclampsia and foetal loss; the prognosis is worse for those who have advanced chronic kidney disease and proteinuria. Here we report the case of a 32-year-old nulliparous woman with chronic hypertension who conceived during an active phase of her IgAN, which had been diagnosed 8 years earlier...
October 2018: Case Reports in Women's Health
Amir Taherkhani, Shiva Kalantari, Afsaneh Arefi Oskouie, Mohsen Nafar, Mohammad Taghizadeh, Koorosh Tabar
Membranous glomerulonephritis (MGN) is one of the most frequent causes of nephrotic syndrome in adults. It is characterized by the thickening of the glomerular basement membrane in the renal tissue. The current diagnosis of MGN is based on renal biopsy and the detection of antibodies to the few podocyte antigens. Due to the limitations of the current diagnostic methods, including invasiveness and the lack of sensitivity of the current biomarkers, there is a requirement to identify more applicable biomarkers...
September 12, 2018: Molecular Medicine Reports
Audrey Uffing, Maria José Pérez-Sáez, Gaetano La Manna, Giorgia Comai, Clara Fischman, Samira Farouk, Roberto Ceratti Manfro, Andrea Carla Bauer, Bruno Lichtenfels, Juliana B Mansur, Hélio Tedesco-Silva, Gianna M Kirsztajn, Anna Manonelles, Oriol Bestard, Miguel Carlos Riella, Silvia Regina Hokazono, Carlos Arias-Cabrales, Elias David-Neto, Carlucci Gualberto Ventura, Enver Akalin, Omar Mohammed, Eliyahu V Khankin, Kassem Safa, Paolo Malvezzi, Michelle Marie O'Shaughnessy, Xingxing S Cheng, Paolo Cravedi, Leonardo V Riella
BACKGROUND: Long-term outcomes in kidney transplantation (KT) have not significantly improved during the past twenty years. Despite being a leading cause of graft failure, glomerular disease (GD) recurrence remains poorly understood, due to heterogeneity in disease pathogenesis and clinical presentation, reliance on histopathology to confirm disease recurrence, and the low incidence of individual GD subtypes. Large, international cohorts of patients with GD are urgently needed to better understand the disease pathophysiology, predictors of recurrence, and response to therapy...
September 12, 2018: BMC Nephrology
Omid Sadeghi-Alavijeh, Scott Henderson, Paul Bass, Terence Cook, Kirsten DeGroot, Alan David Salama
BACKGROUND: Anti-glomerular basement membrane (GBM) antibodies are highly specific for Goodpasture's or anti-GBM disease, in which they are generally directed against the non-collagenous (NC1) domain of the alpha 3 chain of type IV collagen(α3(IV)), and less commonly, toward the α 4(IV) or α 5(IV) chains, which form a triple helical structure in GBM and alveolar basement membrane (ABM). Alterations in the hexameric structure of the NC1 (α3 (IV)), allows novel epitopes to be exposed and an immune response to develop, with subsequent linear antibody deposition along the GBM, leading to a crescentic glomerulonephritis...
September 12, 2018: BMC Nephrology
Guoqin Q Wang, Yipu P Chen, Hong Cheng, Xiaoyi Y Xu, Lijun J Sun, Hongrui R Dong
OBJECTIVES: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder and is characterised by elevated serum IgG4 concentrations and dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-related kidney disease (IgG4-RKD). We report four cases of kidney injury with concurrent IgG4-TIN and crescentic glomerulonephritis confirmed by renal pathology...
August 29, 2018: Clinical and Experimental Rheumatology
H Y Geng, L N Ji, C Y Chen, J Tu, H R Li, R Bao, Y Lin
Objective: To compare the efficacy and safety of mycophenolate mofetil versus cyclosporine A in treating children with primary refractory nephrotic syndrome. Methods: Conducted a prospective randomized controlled clinical trial in 62 pediatric patients (including 44 boys and 18 girls), age ranged from 2.1 to 17.0 years; 32 cases presented with frequently relapsing nephrotic syndrome (FRNS) and 30 cases presented with steroid-resistant nephrotic syndrome (SRNS), who were admitted to department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from October 2013 to October 2015...
September 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
April Ross, Jean Wu, Colin Carlock, William Glass, Ya-Huan Lou
Inter-molecular epitope spreading during autoimmune pathogenesis leads to generation of new pathogenic epitopes on other autoantigens beyond the original one. It raises an important question as whether autoimmunity extends beyond the target tissues if new epitopes are on the molecules shared with other tissues. This study is aimed addressing this question in a rat anti-glomerular basement membrane (GBM) glomerulonephritis model induced by a T cell epitope of glomerulus-specific collagen4α3. We have demonstrated inter-molecular B cell epitope spreading...
2018: PloS One
V Brkovic, M Milinkovic, M Kravljaca, M Lausevic, G Basta-Jovanovic, J Marković-Lipkovski, R Naumovic
Biopsy registries are one of the most important sources of accurate epidemiological data and the clinical presentation of renal diseases. A detailed analysis of clinicopathologic correlations over a period of 20 years (1987-2006) was performed earlier by our centre. The aim of this study was to check the current state and to register possible changes in clinicopathologic findings recorded under better socioeconomical circumstances and new management. Records of 665 renal biopsies performed at our institution were prospectively followed from 2007 to 2014...
October 2018: Pathology, Research and Practice
Sanjeev Sethi, Fernando C Fervenza
A kidney biopsy is done to determine the etiology of the glomerulonephritis (GN) and the severity of the lesion, to identify whether other lesions, related to or not related to the GN, are present on the kidney biopsy and finally to ascertain the extent of chronicity of the GN. The etiology of GN is based on the classification of GN into five groups: immune complex-mediated GN, antineutrophil cytoplasmic antibody (ANCA)-associated GN, anti-glomerular basement membrane (GBM) GN, monoclonal immunoglobulin-mediated GN and C3 glomerulopathy...
August 13, 2018: Nephrology, Dialysis, Transplantation
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