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Goodpasture's Syndrome

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https://www.readbyqxmd.com/read/29124323/-goodpasture-syndrome-ocular-manifestation-in-a-young-man
#1
C Lommatzsch, A Lommatzsch, C Heinz, A Heiligenhaus
Goodpasture syndrome (GS) is a rare organ-specific autoimmune disease that may consist of glomerulonephritis and pulmonary hemorrhage. Only few case reports have been published on ocular symptoms in the context of GS. This is a case report of a 22-year-old male with GS who was referred to our practice because of visual disturbances. The anamnesis revealed pulmonary hemorrhage, acute renal failure and nephrotic syndrome, which was treated by immunosuppressive therapy with additional plasmapheresis. Ophthalmoscopically, bilateral cotton wool spots were found along the vessels and bilateral retinal hemorrhages predominantly at the posterior pole...
November 9, 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/29104431/a-case-of-rare-diffuse-alveolar-hemorrhage-and-review-of-literature
#2
Viviana Scollo, Luca Zanoli, Elisa Russo, Giulio Distefano, Francesco Rapisarda
It is well known that some disorders can cause concomitant kidney dysfunction with lung involvement. These syndromes, characterized by the simultaneous presence of intra-alveolar hemorrhage and acute glomerulonephritis, are caused by numerous and variable disorders. The most frequent are the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and Goodpasture syndrome. A quick discerning of the underlying causes and initiation of adequate treatment is crucial to prevent acute respiratory failure and irreversible loss of renal function...
2017: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29034872/particularities-of-goodpasture-syndrome
#3
Friedrich Lübbecke
No abstract text is available yet for this article.
September 29, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28927772/-antiglomerular-basement-disease-in-children-literature-review-and-therapeutic-options
#4
G Dorval, S Guérin, L Berteloot, S Krid, R Salomon, L Galmiche-Rolland, O Boyer
Antiglomerular basement membrane glomerulonephritis is a rare autoimmune disease characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage (Goodpasture syndrome). The disease is caused by autoantibodies (classically IgGs) directed against the α3 subunit of type IV collagen. This is a rare disease in the adult population and extremely rare in children, with a reported cumulative annual incidence at 1/10(6) people/year. Among scarce reported pediatric cases (n=31), most are girls (M/F sex ratio, 1:4), and the mean age at diagnoses is 9...
September 15, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28863794/immunoadsorption-in-autoimmune-diseases-affecting-the-kidney
#5
REVIEW
Georg Stummvoll, Martin Aringer, Ammon Handisurya, Kurt Derfler
Autoantibodies play an important role in the pathophysiology of renal involvement in systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic vasculitis, and anti-glomerular basement membrane disease (or Goodpasture syndrome). Direct removal of autoantibodies therefore has been tried in various ways, first by plasma exchange. Today, immunoadsorption is the extracorporeal method that most effectively removes (pathogenic) immune complexes and antibodies. Although past data have shown efficacy and biocompatibility of immunoadsorption in (renal) SLE, it is still an experimental and expensive procedure, and evidence from randomized controlled trials is needed...
September 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28853702/familial-antiglomerular-basement-membrane-disease-in-zero-human-leukocyte-antigen-mismatch-siblings%C3%A2
#6
Andrea Angioi, Wisit Cheungpasitporn, Sanjeev Sethi, An S De Vriese, Nicola Lepori, Thomas R Schwab, Fernando C Fervenza
Reported cases of familial Antiglomerular basement membrane (anti-GBM) disease are extremely rare. The single gene mutations that may play a role in the development of familial anti-GBM disease are currently unidentified. While human leukocyte antigen (HLA)-DR15 is known to be associated with an increased risk of anti-GBM disease, HLA types in patients with familial anti-GBM disease have never been reported. We present a case of a 65-year-old woman with rapidly-progressive glomerulonephritis and pulmonary involvement, consistent with Goodpasture's syndrome...
November 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28765179/status-epilepticus-as-the-initial-presentation-of-antibody-negative-goodpasture-s-syndrome
#7
Ingrid Pl Ting, Sanihah Abdul Halim, Azreen Adnan, Hasnan Jaafar
Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28602810/apheresis-to-treat-systemic-vasculitis
#8
Julie Moussi-Frances, Marion Sallée, Noémie Jourde-Chiche
Apheresis has been used in the treatment of severe systemic vasculitides, in conjunction with immunosuppressive therapies, for over 40 years. The aim is to rapidly remove autoantibodies or circulating immune complexes from the plasma. The two main indications at present are vasculitis associated with Antineutrophil Cytoplasmic Antibodies (ANCAs) manifesting as severe renal involvement and/or intraalveolar hemorrhage and antiglomerular basement membrane disease (Goodpasture syndrome). The ongoing PEXIVAS randomized controlled trial is assessing plasmapheresis to treat ANCA-associated vasculitis with or without severe renal involvement or intraalveolar hemorrhage...
June 7, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28573056/antiglomerular-basement-membrane-disease-in-a-pediatric-patient-a-case-report-and-review-of-the-literature
#9
Vimal Master Sankar Raj, Diana Warnecke, Julia Roberts, Sarah Elhadi
Goodpasture's syndrome (GPS) remains a very rare disease entity in the pediatric population characterized by the presence of pulmonary hemorrhage and rapidly evolving glomerulonephritis. We hereby describe the case of a 2-year-old girl who presented with renal failure and was diagnosed with GPS. A brief review of the literature in regard to data on demographics, pathogenesis, clinical features, diagnosis, treatment, and prognosis for renal recovery is also provided.
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28403904/pre-existing-chronic-interstitial-pneumonia-is-a-poor-prognostic-factor-of-goodpasture-s-syndrome-a-case-report-and-review-of-the-literature
#10
REVIEW
Hiroki Tashiro, Koichiro Takahashi, Yuki Ikeda, Saori Uchiumi, Makoto Fukuda, Miyazono Motoaki, Shinya Kimura, Naoko Sueoka-Aragane
BACKGROUND: Goodpasture's syndrome is a rare disease that is characterized by rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage. CASE PRESENTATION: A 71-year-old Japanese man who had chronic interstitial pneumonia was diagnosed as having Goodpasture's syndrome. Both anti-glomerular basement membrane antibody and myeloperoxidase anti-neutrophil cytoplasmic antibody were increased. Despite intensive treatments, including mechanical ventilation, he died from respiratory failure...
April 13, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28356661/anti-glomerular-basement-membrane-disease-case-series-from-a-tertiary-center-in-north-india
#11
D Prabhakar, M Rathi, R Nada, R W Minz, V Kumar, H S Kohli, V Jha, K L Gupta
Anti-glomerular basement (anti-GBM) disease is an uncommon disorder with a bimodal age of presentation. Patients presenting with dialysis-dependent renal failure have poor renal outcomes. There is limited data regarding the clinical presentation and outcomes of anti-GBM disease from India. We conducted this prospective study to analyze the clinical presentation and outcomes of anti-GBM disease at a large tertiary care hospital in North India over 1½ years. Subjects with a biopsy proven anti-GBM disease (light microscopic examination showing crescents and immunofluorescence examination showing linear deposition of IgG) with or without positive anti-GBM antibodies in serum were included in the study and followed-up for at least 12 months...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28260443/outcome-of-renal-transplant-in-recipients-with-vasculitis
#12
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
#13
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/28110970/-plasma-exchange-in-nephrology-indications-and-technique
#14
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...
February 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
#15
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
#16
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
#17
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
#18
REVIEW
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
#19
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
#20
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
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