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Goodpasture's Syndrome

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https://www.readbyqxmd.com/read/28028414/goodpasture-syndrome-diagnosed-one-year-and-a-half-after-the-appearance-of-the-first-symptoms-case-report
#1
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
#2
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/27858870/simultaneous-comprehensive-multiplex-autoantibody-analysis-for-rapidly-progressive-glomerulonephritis
#3
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
#4
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/27498206/plasmapheresis-for-the-treatment-of-kidney%C3%A2-diseases
#5
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
#6
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...
August 2, 2016: 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
#7
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
#8
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/27312909/citrate-anticoagulation-in-tandem-membrane-plasma-exchange-or-immunoadsorption-and-hemodialysis-in-patients-with-immunological-diseases-and-dialysis-dependence
#9
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
#10
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
https://www.readbyqxmd.com/read/27194845/goodpasture-s-disease-in-a-patient-with-kartagener-s-syndrome-an-unusual-association
#11
S B Mandarapu, K K Mukku, S B Raju, S Chandragiri
No abstract text is available yet for this article.
May 2016: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/26876879/the-lung-in-systemic-vasculitis-radiological-patterns-and-differential-diagnosis
#12
REVIEW
Beatrice Feragalli, Cesare Mantini, Marco Sperandeo, Michele Galluzzo, Giovanni Belcaro, Armando Tartaro, Antonio R Cotroneo
The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. The aim of our review is to describe radiographic and high-resolution CT (HRCT) findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Lung disease is a common feature of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides, including granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and microscopic polyangiitis...
2016: British Journal of Radiology
https://www.readbyqxmd.com/read/26792692/-clinicopathological-diagnosis-of-diffuse-alveolar-hemorrhage
#13
F Fang, Y M Li, S T Hu, H T Wang, D G Liu, C Wang
OBJECTIVE: To improve knowledge about the clinical and pathological features of diffuse alveolar hemorrhage (DAH). METHODS: Six cases DAH with intact clinical and pathological data were retrospectively analyzed during the period from May 1999 to May 2015 in Beijing Hospital. There were altogether 2 males and 4 females, with age ranging from 32 to 68 years (mean 58.8 years). Specimens were obtained by autopsy (3 cases), open lung biopsy (2 cases) and renal biopsy (2 cases), including 1 case of open lung biopsy in 2003, renal biopsy in 2012...
January 12, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/26621537/therapeutic-apheresis-in-pregnancy-general-considerations-and-current-practice
#14
REVIEW
Piero Marson, Maria Teresa Gervasi, Tiziana Tison, Anna Colpo, Giustina De Silvestro
It is widely known that pregnancy does not represent a contraindication to therapeutic apheresis (TA) techniques. In fact, since the first experiences of TA in pregnancy for the prevention of hemolytic disease of the newborn, several diseases are at present treated with TA, mainly within 6 clinical categories: (a) TA is a priority and has no alternative equally effective treatment (e.g., thrombotic thrombocytopenic purpura); (b) TA is a priority but there are alternative therapies not contraindicated in pregnancy (e...
December 2015: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/26616334/bullous-pemphigoid-with-a-dual-pattern-of-glomerular-immune-complex-disease
#15
Ewout J Hoorn, Noor E Taams, Tiina Hurskainen, Mahdi Salih, Jan J Weening, Marcel F Jonkman, Hendri H Pas, Marco W J Schreurs
A 75-year-old man presented with a blistering skin disease and nephrotic syndrome. Bullous pemphigoid was diagnosed by linear immunoglobulin G (IgG) and C3 staining along the basement membrane zone of a skin biopsy specimen and by the presence of circulating IgG recognizing the 180-kDa bullous pemphigoid antigen (BP180; type XVII collagen). A kidney biopsy specimen showed endocapillary inflammation without crescents. Direct immunofluorescence showed strong IgG and C3 staining in a combined granular and linear pattern along the glomerular basement membrane...
February 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/26475667/-atypic-hemolytic-uremic-syndrome-taken-for-goodpasture-s-syndrome-a-case-report
#16
Marie Bourgault, Damien Sarret, Pierre Isnard, Marion Rabant, Jacques Labaye
We report the case of a patient suffering from atypical hemolytic uremic syndrome with inaugural intra-alveolar hemorrhage. Clinical features and detection of circulating anti-glomerular basal membrane antibodies first raise the possibility of a Goodpasture syndrome. Renal biopsy allows to correct the diagnosis. Partial remission is obtained thanks to specific care and eculizumab infusions.
December 2015: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/26380476/-goodpasture-s-syndrome-case-reports
#17
Gordana Kotur, Goran Kotur, Ivica Horvatić, Danica Galešić, L Jubanović, Krešimir Galešić
Goodpasture's syndrome is a rare clinical entity characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and the presence of circulating autoantibodies to the glomerular basement membrane (GBM). Autoantibodies bind to reactive epitopes of noncollagenous domain of the collagen type IV alpha-3 chain in glomerular and alveolar basement membranes. Autoantibodies activate the complement cascade resulting in tissue injury by the type II hypersensitivity reaction according to the Coombs and Gell classification of antigen-antibody reactions...
May 2015: Lijec̆nic̆ki Vjesnik
https://www.readbyqxmd.com/read/26304097/infective-endocarditis-in-the-course-of-urosepsis-e-coli-esbl-in-a-patient-with-goodpasture-s-syndrome
#18
Sebastian Spaleniak, Katarzyna Romejko-Ciepielewska, Arkadiusz Lubas, Robert Ryczek, Stanisław Niemczyk
No abstract text is available yet for this article.
2015: Kardiologia Polska
https://www.readbyqxmd.com/read/26167308/severe-uncompensated-metabolic-alkalosis-due-to-plasma-exchange-in-a-patient-with-pulmonary-renal-syndrome-a-clinician-s-challenge
#19
Mohsin Ijaz, Naeem Abbas, Dmitry Lvovsky
Metabolic alkalosis secondary to citrate toxicity from plasma exchange is very uncommon in patients with normal renal function. In patients with advanced renal disease this can be a fatal event. We describe a case of middle-aged woman with Goodpasture's syndrome treated with plasma exchange who developed severe metabolic alkalosis. High citrate load in plasma exchange fluid is the underlying etiology. Citrate metabolism generates bicarbonate and once its level exceeds the excretory capacity of kidneys, the severe metabolic alkalosis ensues...
2015: Case Reports in Critical Care
https://www.readbyqxmd.com/read/26069804/serum-antibody-negative-goodpasture-syndrome-with-delta-granule-pool-storage-deficiency-and-eosinophilia
#20
Ashleigh Kussman, Amira Gohara
Goodpasture syndrome is a rare, life-threatening autoimmune disease characterized by a triad of rapidly progressive glomerulonephritis, a hemorrhagic pulmonary condition and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. The antibodies initiate destruction of the kidney glomeruli, resulting in a focal necrotizing glomerulitis, which may progress rapidly to renal failure. Autoantibody-mediated damage of alveolar basement membranes leads to diffuse pulmonary hemorrhage, which in some cases may be severe enough to cause respiratory failure...
December 2012: Clinical Kidney Journal
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