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https://www.readbyqxmd.com/read/28314809/goodpasture-s-disease-with-late-presentation-of-renal-abnormality-and-anti-gbm-autoantibody
#1
Emily Ann Boardman, Sameira Sohail, Rajesh Yadavilli
Goodpasture's disease without circulating autoantibodies is a rare presentation of a rare diagnosis. We present the case of a man aged 17 years who had 3 hospital admissions over a 2-month period with haemoptysis and shortness of breath. Throughout his first 2 admissions, his renal function was normal and anti-glomerular basement membrane (GBM) antibodies were negative. CT pulmonary angiogram during his second admission revealed florid and diffuse alveolar infiltrates. However, high-resolution CT chest performed 4 weeks later showed complete resolution of these changes...
March 17, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28296059/the-pathogenicity-of-t-cell-epitopes-on-human-goodpasture-antigen-and-its-critical-amino-acid-motif
#2
Shui-Yi Hu, Qiu-Hua Gu, Jia Wang, Miao Wang, Xiao-Yu Jia, Zhao Cui, Ming-Hui Zhao
Goodpasture antigen, the non-collagenous domain of α3 chain of type IV collagen [α3(IV)NC1], is the target antigen of anti-glomerular basement membrane (GBM) antibodies. The pathogenicity of T cell epitopes is not elucidated clearly. In this study, we aim to define the nephritogenic T cell epitopes and its critical amino acid residues. Twenty-four overlapping linear peptides were synthesized covering the whole sequence of human α3(IV)NC1. Wistar-Kyoto rats were immunized with linear peptides, and experimental autoimmune glomerulonephritis was evaluated...
March 10, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28260443/outcome-of-renal-transplant-in-recipients-with-vasculitis
#3
Samia Barbouch, Meriam Hajji, Raja Aoudia, Monther Ounissi, Asma Zammouri, Rym Goucha, Fathi Ben Hamida, Mohammed Mongi Bacha, Ezzedine Abderrahim, Taieb Ben Abdallah
OBJECTIVES: End-stage renal disease develops in a high percentage of patients with vasculitis, in whom kidney transplant has become a therapeutic option. However, limited data are available on the prognosis and outcomes after kidney transplant in these patients. We aimed to compare the long-term graft survival and graft function in 8 renal transplant recipients with vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, Goodpasture syndrome, and Henoch-Schonlein purpura) with the other kidney recipients at a single center...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28249989/associations-between-specific-autoimmune-diseases-and-subsequent-dementia-retrospective-record-linkage-cohort-study-uk
#4
Clare J Wotton, Michael J Goldacre
OBJECTIVE: To determine whether hospital admission for autoimmune disease is associated with an elevated risk of future admission for dementia. METHODS: Retrospective, record-linkage cohort study using national hospital care and mortality administrative data, 1999-2012. Cohorts of people admitted to hospital with a range of autoimmune diseases were constructed, along with a control cohort, and followed forward in time to see if they developed dementia. 1 833 827 people were admitted to hospital with an autoimmune disease; the number of people in cohorts for each autoimmune disease ranged from 1019 people in the Goodpasture's syndrome cohort, to 316 043 people in the rheumatoid arthritis cohort...
March 1, 2017: Journal of Epidemiology and Community Health
https://www.readbyqxmd.com/read/28152559/t-cell-responses-to-peptides-of-goodpasture-autoantigen-in-patients-with-anti-glomerular-basement-membrane-disease
#5
Shui-Yi Hu, Xiao-Yu Jia, Qiu-Hua Gu, Chong-Yan Yu, Xu-Yang Cheng, Qi-Zhuang Jin, Fu-de Zhou, Zhao Cui, Ming-Hui Zhao
AIM: Cell-mediated autoimmunity, especially autoreactive T cells, is crucial in the initiation of anti-glomerular membrane (GBM) disease. Epitopes for T cells on Goodpasture autoantigen are not fully defined. This study investigated T cell epitopes in anti-GBM patients, aiming to identify the epitopes and their clinical significance. METHODS: Peripheral blood mononuclear cells (PBMC) were collected from 13 patients with anti-GBM disease. Twenty-four overlapping linear peptides were synthesized covering the whole sequence of human α3(IV)NC1...
February 2, 2017: Nephrology
https://www.readbyqxmd.com/read/28110970/-plasma-exchange-in-nephrology-indications-and-technique
#6
Christophe Ridel, Sébastien Kissling, Laurent Mesnard, Alexandre Hertig, Éric Rondeau
Plasma exchange is a non-selective apheresis technique that can be performed by filtration or centrifugation allowing rapid purification of high molecular weight pathogens. An immunosuppressive treatment is generally associated to reduce the rebound effect of the purified substance. Substitution solutes such as human albumin and macromolecules are needed to compensate for plasma extraction. Compensation by viro-attenuated plasma is reserved solely for the treatment of thrombotic microangiopathies or when there is a risk of bleeding, because this product is very allergenic and expensive...
January 16, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28028414/goodpasture-syndrome-diagnosed-one-year-and-a-half-after-the-appearance-of-the-first-symptoms-case-report
#7
Jagoda Stojkovikj, Sead Zejnel, Biljana Gerasimovska, Vesna Gerasimovska, Dragana Stojkovic, Martin Trajkovski, Irina Angelovska, Angela Debreslioska, Smilko Jovanovski
BACKGROUND: Goodpasture syndrome was originally described as an association of alveolar haemorrhage and glomerulonephritis. It occurs when the immune system attacks and destroys healthy body tissue. AIM: We are presenting a patient with a clinical picture of pulmonary haemorrhage and glomerulonephritis, which is diagnosed by renal biopsy. CASE PRESENTATION: His illness began a year and a half before being diagnosed. In that period he had occasional exacerbations...
December 15, 2016: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/27999694/a-case-of-pulmonary-renal-syndrome-leading-to-the-diagnosis-of-legionnaires-disease
#8
Erasmia Sabani, Pantelis A Sarafidis, Antonios Lazaridis, Theodora Kouloukourgiotou, Konstantinos Stylianou, Afroditi Pantzaki, Aikaterini Papagianni, Georgios Efstratiadis
We report a case of a 51-year-old Caucasian man referred at our department due to acute renal failure (ARF) complicating respiratory failure during hospitalization in a regional hospital. The patient was previously started on steroids due to the suspicion of rapidly progressive glomerulonephritis (RPGN) in the context of Goodpasture syndrome. However, clinical and laboratory findings did not support this diagnosis; instead a careful evaluation limited differential diagnosis of the renal insult to acute tubular necrosis or acute interstitial nephritis (AIN) following respiratory infection...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27981771/goodpasture-disease-as-a-consequence-of-melioidosis
#9
D Mackintosh, M Mantha, K Oliver
We describe a case of anti-glomerular basement membrane (GBM) antibody-mediated disease in association with concomitant Burkholderia pseudomallei (melioidosis) bacteraemia. The temporal profile of the illness and initial absence of circulating anti-GBM antibodies, in light of the subsequent definitive histological diagnosis of anti-GBM disease, makes this case interesting and unusual. Additionally, there have been no prior case reports suggesting melioidosis as a cause of biopsy-proven glomerulonephritis.
December 2016: Internal Medicine Journal
https://www.readbyqxmd.com/read/27899391/atypical-presentation-of-anti-gbm-nephritis-in-a-90-year-old-patient
#10
Julie Omolola Okiro, Chaudhry Adeel Ebad, Amjad Zaman Khan
A 90-year-old woman who had bloody diarrhoea, nausea, weakness and reduced urine output was found to have acute kidney injury. Her inflammatory markers were raised and her chest X-ray suggested an inflammatory process. She was initially suspected to have acute kidney injury secondary to dehydration and sepsis but when her autoimmune screen returned positive for antiglomerular basement membrane antibodies our diagnosis and management strategy was reconsidered. This is a case report of Goodpasture disease presenting in an elderly patient...
November 29, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27858870/simultaneous-comprehensive-multiplex-autoantibody-analysis-for-rapidly-progressive-glomerulonephritis
#11
Mandy Sowa, Barbara Trezzi, Rico Hiemann, Peter Schierack, Kai Grossmann, Juliane Scholz, Valentina Somma, Renato Alberto Sinico, Dirk Roggenbuck, Antonella Radice
Rapidly progressive glomerulonephritis (RPGN) is mainly caused by anti-glomerular basement membrane (GBM) antibody-mediated glomerulonephritis, immune-complex or anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides and leads to rapid loss of renal function. Detection of ANCA and autoantibodies (autoAbs) to GBM and dsDNA enables early diagnosis and appropriate treatment of RPGN aiding in preventing end-stage renal disease.Determination of ANCA on neutrophils (ANCA) as well as autoAbs to myeloperoxidase (MPO-ANCA), proteinase 3 (PR3-ANCA), GBM, and dsDNA was performed by the novel multiplex CytoBead technology combining cell- and microbead-based autoAb analyses by automated indirect immunofluorescence (IIF)...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27829255/-pulmonary-manifestations-of-vasculitis
#12
S von Vietinghoff
The variable symptoms and signs of pulmonary vasculitis are a diagnostic and therapeutic challenge. Vasculitis should be considered in rapidly progressing, severe and unusual manifestations of pulmonary disease. Clinical examination of other organ systems typically affected by vasculitis such as skin and kidney and autoantibody measurements are complementary approaches to manage this situation. Pulmonary involvement is common in small vessel vasculitis including anti-GBM disease (Goodpasture syndrome) and the ANCA-associated vasculitides...
November 2016: Pneumologie
https://www.readbyqxmd.com/read/27619726/embryo-implantation-triggers-dynamic-spatiotemporal-expression-of-the-basement-membrane-toolkit-during-uterine-reprogramming
#13
Celestial R Jones-Paris, Sayan Paria, Taloa Berg, Juan Saus, Gautam Bhave, Bibhash C Paria, Billy G Hudson
Basement membranes (BMs) are specialized extracellular scaffolds that influence behaviors of cells in epithelial, endothelial, muscle, nervous, and fat tissues. Throughout development and in response to injury or disease, BMs are fine-tuned with specific protein compositions, ultrastructure, and localization. These features are modulated through implements of the BM toolkit that is comprised of collagen IV, laminin, perlecan, and nidogen. Two additional proteins, peroxidasin and Goodpasture antigen-binding protein (GPBP), have recently emerged as potential members of the toolkit...
January 2017: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/27498206/plasmapheresis-for-the-treatment-of-kidney%C3%A2-diseases
#14
REVIEW
William F Clark, Shih-Han S Huang, Michael W Walsh, Myriam Farah, Ainslie M Hildebrand, Jessica M Sontrop
The purpose of this review is to examine the evidence supporting the application of plasma exchange in renal disease. Our review focuses on the following 6 most common renal indications for plasma exchange based on 2014 registry data from the Canadian Apheresis Group: (i) thrombotic thrombocytopenic purpura (TTP)/hemolytic uremic syndrome; (ii) renal transplantation, (iii) anti-neutrophil cytoplasm antibodies-associated vasculitis, (iv) cryoglobulinemia, (v) focal segmental glomerulosclerosis, and (vi) Goodpasture syndrome...
November 2016: Kidney International
https://www.readbyqxmd.com/read/27496347/basement-membranes-and-autoimmune-diseases
#15
REVIEW
Mary H Foster
Basement membrane components are targets of autoimmune attack in diverse diseases that destroy kidneys, lungs, skin, mucous membranes, joints, and other organs in man. Epitopes on collagen and laminin, in particular, are targeted by autoantibodies and T cells in anti-glomerular basement membrane glomerulonephritis, Goodpasture's disease, rheumatoid arthritis, post-lung transplant bronchiolitis obliterans syndrome, and multiple autoimmune dermatoses. This review examines major diseases linked to basement membrane autoreactivity, with a focus on investigations in patients and animal models that advance our understanding of disease pathogenesis...
January 2017: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/27459964/goodpasture-s-syndrome-with-absence-of-circulating-anti-glomerular-basement-membrane-antibodies-a-case-report
#16
Rui Fernandes, Sara Freitas, Pedro Cunha, Gloria Alves, Jorge Cotter
BACKGROUND: Goodpasture's syndrome, a rare disease, is an organ-specific autoimmune disease mediated by anti-glomerular basement membrane antibodies. Its pathology is characterized by crescentic glomerulonephritis with linear immunofluorescent staining for immunoglobulin G on the glomerular basement membrane. Although rare, a few cases with absence of circulating anti-glomerular membrane antibodies have been described. CASE PRESENTATION: The objective of this clinical case report is to describe and discuss a case of a 27-year-old white man who was hospitalized with a 1-year history of weight loss and a 1-month history of hemoptysis, with aggravation  the day before, having developed dyspnea and cough in the previous 24 hours...
July 27, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27450516/uncommon-structural-motifs-dominate-the-antigen-binding-site-in-human-autoantibodies-reactive-with-basement-membrane-collagen
#17
Mary H Foster, Elizabeth S Buckley, Benny J Chen, Kwan-Ki Hwang, Amy G Clark
Autoantibodies mediate organ destruction in multiple autoimmune diseases, yet their origins in patients remain poorly understood. To probe the genetic origins and structure of disease-associated autoantibodies, we engrafted immunodeficient mice with human CD34+ hematopoietic stem cells and immunized with the non-collagenous-1 (NC1) domain of the alpha3 chain of type IV collagen. This antigen is expressed in lungs and kidneys and is targeted by autoantibodies in anti-glomerular basement membrane (GBM) nephritis and Goodpasture syndrome (GPS), prototypic human organ-specific autoimmune diseases...
August 2016: Molecular Immunology
https://www.readbyqxmd.com/read/27335377/optimizing-the-translational-value-of-animal-models-of-glomerulonephritis-insights-from-recent-murine-prototypes
#18
REVIEW
Mary H Foster
Animal models are indispensable for the study of glomerulonephritis, a group of diseases that destroy kidneys but for which specific therapies do not yet exist. Novel interventions are urgently needed, but their rational design requires suitable in vivo platforms to identify and test new candidates. Animal models can recreate the complex immunologic microenvironments that foster human autoimmunity and nephritis and provide access to tissue compartments not readily examined in patients. Study of rat Heymann nephritis identified fundamental disease mechanisms that ultimately revolutionized our understanding of human membranous nephropathy...
September 1, 2016: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/27312909/citrate-anticoagulation-in-tandem-membrane-plasma-exchange-or-immunoadsorption-and-hemodialysis-in-patients-with-immunological-diseases-and-dialysis-dependence
#19
Rafael Ponikvar, Jakob Gubenšek, Jadranka Buturović Ponikvar
In 1996 we performed tandem membrane plasma exchange-hemodialysis in a 3-year-old girl and tandem immunoadsorption-hemodialysis with citrate as the only anticoagulant in a patient with Goodpasture's syndrome. In the present study, we evaluated the feasibility, efficacy and safety of 24 tandem plasma exchange/immunoadsorption hemodialysis procedures in four different circuit setups with citrate as the only anticoagulant. In two setups, the tandem procedures were connected in series (plasma exchange hemodialysis and immunoadsorption hemodialysis), while in the other two setups they were in parallel (plasma exchange hemodialysis with independent blood circuits and plasma exchange hemodialysis with independent arterial blood lines, but with a common return line)...
June 2016: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/27267459/outcomes-of-patients-with-goodpasture-syndrome-a-nationwide-cohort-based-study-from-the-french-society-of-hemapheresis
#20
Antoine Huart, Anne-Gaëlle Josse, Dominique Chauveau, Jean-Michel Korach, Farhad Heshmati, Eric Bauvin, Olivier Cointault, Nassim Kamar, David Ribes, Jacques Pourrat, Stanislas Faguer
The overall and renal outcomes of patients with Goodpasture syndrome (GS), a rare autoimmune disorder characterized by circulating anti-GBM antibodies and rapidly progressive glomerulonephritis and/or pulmonary hemorrhage, have mostly been reported in small-sized cohorts or by aggregating patients receiving a variety of therapies that include aggressive (i.e., combined plasma exchanges, corticosteroids, and cyclophosphamide) and less aggressive (i.e., either plasma exchanges or immunosuppressive drugs, or no treatment)...
September 2016: Journal of Autoimmunity
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