684 results match your criteria Goodpasture Syndrome


Coryneform Bacteria in Granulomatous Lobular Mastitis: Morphological Diagnosis in Breast Biopsies.

Int J Surg Pathol 2018 Nov 29:1066896918815580. Epub 2018 Nov 29.

1 Pontificia Universidad Católica de Chile, Santiago, Chile.

Granulomatous lobular mastitis is a rare disease whose origin is still unknown and shows an increase in its frequency. Morphological, microbiological, and molecular biology studies have linked this disease to lipophilic and fastidious corynebacteria, suggesting its possible infectious etiology. This series describes and reviews in detail the distinctive morphological characteristics of the bacteria present in the granulomas of this disease, the usefulness of histochemical techniques for their identification, and our proposal for a tissue quantification score for the bacteria. Read More

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November 2018

Mechanisms and functions of IL-17 signaling in renal autoimmune diseases.

Mol Immunol 2018 Dec 15;104:90-99. Epub 2018 Nov 15.

III. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany; Section of Translational Immunology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. Electronic address:

Immune-mediated glomerular diseases (glomerulonephritis) encompass a heterogeneous collection of diseases that cause inflammation within the glomerulus and other renal compartments with significant morbidity and mortality. In general, CD4 T cells orchestrate the immune response and play a unique role in autoimmune and chronic inflammatory diseases. In particular, the characterization of a distinct, IL-17 cytokines producing CD4 T cell subset named T17 cells has significantly advanced the current understanding of the pathogenic mechanisms of organ-specific immunity. Read More

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December 2018
2 Reads

Unicellular ancestry and mechanisms of diversification of Goodpasture-antigen binding protein.

J Biol Chem 2018 Oct 30. Epub 2018 Oct 30.

Center for Matrix Biology, Vanderbilt University Medical Center, United States.

The emergence of the basement membrane (BM), a specialized form of extracellular matrix, was essential in the unicellular transition to multicellularity. Yet, the mechanism is unknown.  Goodpasture antigen-binding protein (GPBP), aBM protein, was uniquely poised to play diverse roles in this transition owing to its multiple isoforms (GPBP-1, -2 and -3) with varied intracellular and extracellular functions (ceramide trafficker and protein kinase). Read More

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October 2018

Peroxidasin-a Novel Autoantigen in Anti-GBM Disease?

J Am Soc Nephrol 2018 Nov 12;29(11):2605-2607. Epub 2018 Oct 12.

Centre for Inflammatory Disease, Department of Medicine, Imperial College London, London, United Kingdom.

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November 2018
4 Reads

Inhibitory Anti-Peroxidasin Antibodies in Pulmonary-Renal Syndromes.

J Am Soc Nephrol 2018 Nov 2;29(11):2619-2625. Epub 2018 Oct 2.

Department of Nephrology and Hypertension,

Background: Goodpasture syndrome (GP) is a pulmonary-renal syndrome characterized by autoantibodies directed against the NC1 domains of collagen IV in the glomerular and alveolar basement membranes. Exposure of the cryptic epitope is thought to occur disruption of sulfilimine crosslinks in the NC1 domain that are formed by peroxidasin-dependent production of hypobromous acid. Peroxidasin, a heme peroxidase, has significant structural overlap with myeloperoxidase (MPO), and MPO-ANCA is present both before and at GP diagnosis in some patients. Read More

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November 2018
4 Reads

Red to green pigment change noted in plasma of patient undergoing therapeutic plasma exchange for Goodpasture syndrome.

Transfusion 2018 Oct;58(10):2270-2271

University of Texas Medical Branch, Department of Pathology, Galveston, Texas.

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October 2018
5 Reads

Anti-Glomerular Basement Membrane Disease.

Rheum Dis Clin North Am 2018 11 7;44(4):651-673. Epub 2018 Sep 7.

Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, Commonwealth Building, Hammersmith Hospital Campus, Du Cane Road, London W12 ONN, UK; Vasculitis Clinic, Imperial College Healthcare NHS Trust, Hammersmith Hospital, Du Cane Road, London W12 0HS, UK. Electronic address:

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. Read More

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November 2018
1 Read

A rare fatal complication of bleeding immediately following polytetrafluoroethylene arteriovenous grafting in a lady with Goodpasture syndrome.

Hemodial Int 2018 Sep 25. Epub 2018 Sep 25.

Madras Medical Mission Hospital, Chennai, 600 037, India.

We report a 28-year-old female with no history of allergies and recent onset of Goodpasture syndrome who developed life-threatening bleeding immediately after placement of a polytetrafluoroethylene (PTFE) graft as an access for hemodialysis in the left upper limb by an experienced vascular surgeon. In spite of transfusing fresh frozen plasma, packed cells and cryoprecipitate, her prothrombin time (PT), activated partial thromboplastin time and international normalized ratio became progressively worse which were normal at the beginning of the surgery. She had profound hypotension and succumbed within 8 hours. Read More

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September 2018
3 Reads

Mid-ventricle Takotsubo in a Patient with Goodpasture Syndrome.

Cureus 2018 Jul 17;10(7):e2990. Epub 2018 Jul 17.

Cardiovascular Division, University of Miami Miller School of Medicine/Jackson Memorial Hospital, Miami, USA.

Takotsubo syndrome (TTS) is characterized by transient, regional systolic dysfunction of the left ventricle, often mimicking acute coronary syndrome. Atypical variants of this syndrome with mid-ventricular, basal, and focal wall motion patterns are increasingly diagnosed and show different clinical features compared to typical TTS. Even though TTS was generally considered a benign condition, continuous and strict monitoring is necessary to diagnose potentially life-threating complications. Read More

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July 2018
2 Reads

Use of rituximab as an induction therapy in anti-glomerular basement-membrane disease.

BMC Nephrol 2018 Sep 20;19(1):241. Epub 2018 Sep 20.

Service de Néphrologie, Hémodialyse, Aphérèses et Transplantation, CHU Grenoble-Alpes, Avenue Maquis du Grésivaudan, 38700, La Tronche, France.

Background: Anti-glomerular basement-membrane (anti-GBM) disease (or Goodpasture disease) is characterized by severe kidney and lung involvement. Prognoses have improved with treatments that combine plasma exchange and immunosuppressive drugs. However, patients with severe renal involvement can have poor renal outcomes and cyclophosphamide can cause significant complications. Read More

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September 2018
1 Read

Preserving fertility in an unconscious patient with Goodpasture syndrome-medicolegal and ethical aspects.

J Intensive Care 2018 23;6:40. Epub 2018 Jul 23.

1Medical Intensive Care Unit, University Hospital of Zurich, Rämistrasse 100, 8091 Zurich, Switzerland.

Background: Every day in the ICU, legal issues arise while treating sedated, unconscious, and legally incapacitated patients. Whenever a life-saving treatment cannot be discussed in a timely manner with an unconscious patient, doctors are required by law to act according to the substituted judgment standard. However, if it is not survival that is at stake, but conservation of reproduction and the potential side effects are significant, the decision-making process becomes much more difficult. Read More

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July 2018
1 Read

Routinely used immunoassays do not detect circulating anti-GBM antibodies against native NC1 hexamer and EA epitope of the α3 chain of type IV collagen.

Eur J Immunol 2018 Jun 2;48(6):1082-1084. Epub 2018 May 2.

Laboratoire d'Immunologie, Pôle de Biologie, Centre Hospitalier Universitaire Grenoble Alpes, Grenoble, Cedex 9, France.

Detection of circulating anti-GBM antibodies has a key role for the diagnosis of Goodpasture syndrome but immunoassays using purified or recombinant alpha3(IV)NC1 as antigen do not recognize all anti-GBM antibodies. We show that anti-GBM antibodies directed against epitopes in their native conformation or cryptic epitopes are detected by indirect immunofluorescence. Read More

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June 2018
4 Reads

Selective targeting of collagen IV in the cancer cell microenvironment reduces tumor burden.

Oncotarget 2018 Feb 19;9(13):11020-11045. Epub 2018 Jan 19.

FibroStatin, Parc Científic Universitat de València, Paterna 46980, Spain.

Goodpasture antigen-binding protein (GPBP) is an exportable Ser/Thr kinase that induces collagen IV expansion and has been associated with chemoresistance following epithelial-to-mesenchymal transition (EMT). Here we demonstrate that cancer EMT phenotypes secrete GPBP (mesenchymal GPBP) which displays a predominant multimeric oligomerization and directs the formation of previously unrecognized mesh collagen IV networks (mesenchymal collagen IV). Yeast two-hybrid (YTH) system was used to identify a SHCIE motif critical for multimeric GPBP assembly which then facilitated design of a series of potential peptidomimetics. Read More

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February 2018
1 Read

The treatment of ANCA-associated rapidly-progressive glomerulonephritis and Goodpasture syndrome with therapeutic apheresis.

Authors:
Suheyla Apaydin

Transfus Apher Sci 2018 Feb 20;57(1):8-12. Epub 2018 Feb 20.

University of Health Sciences, Medical Faculty, Internal Medicine, Bakirkoy Dr. Sadi Konuk Training and Research Center, Nephrology Department, Istanbul, Turkey. Electronic address:

Therapeutic plasma aphresis (plasmapheresis) is one form of treatment that is frequently used in practice of Nephrology. Plasmapheresis is the most important part of the therapies for Goodpasture's syndrome and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis which are causes of rapidly progressive glomerulonephritis. The reason why the effectiveness of plasmapheresis therapy cannot be clearly demonstrated in renal involvement in these diseases is that it does not appear to be possible to recruit an adequate number of patients and plasmapheresis is not effective in advanced disease if early treatment is not initiated. Read More

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February 2018
5 Reads

Goodpasture's syndrome in early pregnancy: A case report.

Exp Ther Med 2018 Jan 1;15(1):407-411. Epub 2017 Nov 1.

Guizhou Maternal and Child Health Hospital, Guiyang, Guizhou 550000, P.R. China.

Goodpasture's syndrome (GPS) presenting during pregnancy is extremely rare and patients exhibiting hemoptysis and renal dysfunction in early pregnancy are uncommon. The present study reports the case of a 17-year-old diagnosed with GPS during the thirteenth week of pregnancy. Prompt recognition and immediate treatment (steroids and plasma exchange) was initiated. Read More

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January 2018
20 Reads

[A giant cell arteritis revealing a Goodpasture's syndrome].

Nephrol Ther 2018 Apr 28;14(2):105-108. Epub 2017 Dec 28.

Service de médecine interne, centre hospitalier Saint-Joseph-Saint-Luc, 20, quai Claude-Bernard, 69007 Lyon, France.

Goodpasture's syndrome is a triad of anti-glomerular basement membrane (anti-GBM) circulating antibodies, glomerulonephritis and pulmonary hemorrhage. We reported a 65-year-old woman with headaches, asthenia and weight loss. Giant cell arteritis was confirmed by temporal artery biopsy. Read More

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April 2018
4 Reads

Rapidly progressive glomerulonephritis due to anti-glomerular basement membrane disease accompanied by IgA nephropathy: An unusual association.

Saudi J Kidney Dis Transpl 2017 Nov-Dec;28(6):1404-1407

Department of Nephrology, Government Stanley Hospital, Chennai, Tamil Nadu, India.

Anti-glomerular basement membrane (anti-GBM) disease is a systemic autoimmune disorder characterized by circulating IgG antibodies (rarely IgA and IgM) to the carboxyterminal, noncollagenous 1 (NC1) domain of type IV collagen of GBM also known as Goodpasture antigen. Patients typically present with rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage in the presence of which it is referred to as Goodpasture's disease. Anti-GBM disease has been reported to coexist with pauci-immune antineutrophil cytoplasmic autoantibody-positive glomerulonephritis and membranous glomerulopathy. Read More

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December 2017
36 Reads

Predicting Outcome in Patients with Anti-GBM Glomerulonephritis.

Clin J Am Soc Nephrol 2018 Jan 21;13(1):63-72. Epub 2017 Nov 21.

Departments of Pathology and.

Background And Objectives: Large studies on long-term kidney outcome in patients with anti-glomerular basement membrane (anti-GBM) GN are lacking. This study aimed to identify clinical and histopathologic parameters that predict kidney outcome in these patients.

Design, Setting, Participants, & Measurements: This retrospective analysis included a total of 123 patients with anti-GBM GN between 1986 and 2015 from six centers worldwide. Read More

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January 2018
39 Reads

[Goodpasture syndrome: ocular manifestation in a young man].

Ophthalmologe 2018 Sep;115(9):761-764

Augenzentrum, St. Franziskus Krankenhaus Münster, Hohenzollernring 74, 48145, Münster, Deutschland.

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. Read More

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September 2018
8 Reads

A Case of Rare Diffuse Alveolar Hemorrhage and Review of Literature.

Clin Med Insights Case Rep 2017 16;10:1179547617726077. Epub 2017 Aug 16.

Section of Nephrology, Division of Internal Medicine, Department of Clinical and Experimental Medicine, Policlinico Universitario, University of Catania, Catania, Italy.

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. Read More

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August 2017
12 Reads

Particularities of Goodpasture Syndrome.

Dtsch Arztebl Int 2017 09;114(39):662

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September 2017
6 Reads

[Antiglomerular basement disease in children: Literature review and therapeutic options].

Arch Pediatr 2017 Oct 18;24(10):1019-1028. Epub 2017 Sep 18.

Service de néphrologie pédiatrique, Marhea, hôpital Necker-Enfants-Malades, 149, rue de Sèvres, 75015 Paris, France; Institut Imagine, université Paris Descartes-Sorbonne Paris Cité, , 75006 Paris, France.

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 people/year. Read More

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October 2017
13 Reads

Smoking cessation and outcome after ischemic stroke or TIA.

Neurology 2017 Oct 8;89(16):1723-1729. Epub 2017 Sep 8.

From the Yale School of Medicine (K.A.E., C.M.V., L.H.Y., S.E.I., B.G., W.N.K.), New Haven, CT; University of Western Ontario (J.D.S.), London, Canada; Maine Medical Center (M.G.), Portland; Fred Hutchinson Cancer Research Center (P.D.G.), Seattle, WA; University of Newcastle Upon Tyne (A.D.), Newcastle, UK; and Alpert Medical School of Brown University (K.L.F.), Providence, RI.

Objective: To assess whether smoking cessation after an ischemic stroke or TIA improves outcomes compared to continued smoking.

Methods: We conducted a prospective observational cohort study of 3,876 nondiabetic men and women enrolled in the Insulin Resistance Intervention After Stroke (IRIS) trial who were randomized to pioglitazone or placebo within 180 days of a qualifying stroke or TIA and followed up for a median of 4.8 years. Read More

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October 2017
6 Reads

Immunoadsorption in Autoimmune Diseases Affecting the Kidney.

Semin Nephrol 2017 09;37(5):478-487

Department of Nephrology, Medical University of Vienna, Vienna, Austria.

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. Read More

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September 2017
27 Reads

Apheresis to treat systemic vasculitis.

Joint Bone Spine 2018 Mar 7;85(2):177-183. Epub 2017 Jun 7.

Centre de néphrologie et transplantation rénale, hôpital de la Conception, AP-HM, 147, boulevard Baille, 13005 Marseille, France; Faculté de médecine, Aix-Marseille université, 27, boulevard Jean-Moulin, 13005 Marseille, France; Faculté de pharmacie, Aix-Marseille université, UMRS_1076 Vascular Research Center of Marseille, 27, boulevard Jean-Moulin, 13005 Marseille, France. Electronic address:

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). Read More

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March 2018
22 Reads

The critical amino acids of a nephritogenic epitope on human Goodpasture autoantigen for binding to HLA-DRB1*1501.

Mol Immunol 2017 08 29;88:1-9. Epub 2017 May 29.

Renal Division, Peking University First Hospital, Beijing 100034, PR China; Institute of Nephrology, Peking University, Beijing 100034, PR China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing 100034, PR China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing 100034, PR China; Peking-Tsinghua Center for Life Sciences, Beijing 100871, PR China.

Background: Anti-GBM disease is caused by autoimmunity to Goodpasture antigen on α3(IV)NC1 and had strong associations with HLA-DRB1*1501. Previous studies identified α3 (P14: TDIPPCPHGWISLWKGFSFIMF) as a T cell epitope. The present study was aimed to investigate the binding capacity of P14 to HLA-DRB1*1501 and the critical amino acids for this binding. Read More

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August 2017
20 Reads

Antibodies against linear epitopes on Goodpasture autoantigen in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis.

Clin Rheumatol 2017 Sep 26;36(9):2087-2094. Epub 2017 May 26.

Renal Division, Department of Medicine; Institute of Nephrology, Key Laboratory of Renal Disease, Ministry of Health of China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Peking University First Hospital, Beijing, China.

In a substantial number of patients with crescentic glomerulonephritis, both anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) are detected simultaneously. ANCA is presumed to be the initial event but the mechanism is unknown. In the present study, we investigated the antibodies against linear epitopes on Goodpasture autoantigen in sera from patients with ANCA-associated vasculitis, aiming to reveal the mechanisms of the coexistence of the two kinds of autoantibodies. Read More

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September 2017
20 Reads

Autoimmunity: HLA-mediated protection in Goodpasture disease.

Authors:
Ellen F Carney

Nat Rev Rheumatol 2017 Jul 18;13(7):387. Epub 2017 May 18.

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July 2017
9 Reads

Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients.

Kidney Int 2017 09 12;92(3):693-702. Epub 2017 May 12.

Renal and Vascular Inflammation Section, Department of Medicine, Imperial College London, London, UK.

Co-presentation with both ANCA and anti-GBM antibodies is thought to be relatively rare. Current studies of such 'double-positive' cases report small numbers and variable outcomes. To study this further we retrospectively analyzed clinical features and long-term outcomes of a large cohort of 568 contemporary patients with ANCA-associated vasculitis, 41 patients with anti-GBM disease, and 37 double-positive patients with ANCA and anti-GBM disease from four European centers. Read More

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September 2017
29 Reads

Autoimmunity: HLA-mediated protection in Goodpasture disease.

Authors:
Ellen F Carney

Nat Rev Nephrol 2017 07 15;13(7):381. Epub 2017 May 15.

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July 2017
6 Reads

Dominant protection from HLA-linked autoimmunity by antigen-specific regulatory T cells.

Nature 2017 05 3;545(7653):243-247. Epub 2017 May 3.

Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Clayton, Victoria 3168, Australia.

Susceptibility and protection against human autoimmune diseases, including type I diabetes, multiple sclerosis, and Goodpasture disease, is associated with particular human leukocyte antigen (HLA) alleles. However, the mechanisms underpinning such HLA-mediated effects on self-tolerance remain unclear. Here we investigate the molecular mechanism of Goodpasture disease, an HLA-linked autoimmune renal disorder characterized by an immunodominant CD4 T-cell self-epitope derived from the α3 chain of type IV collagen (α3). Read More

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May 2017
31 Reads

Diagnostic and management challenges in Goodpasture's (anti-glomerular basement membrane) disease.

Nephrol Dial Transplant 2018 Feb;33(2):196-202

Centre for Nephrology, Division of Medicine, University College London, Royal Free Hospital, London, UK.

Goodpasture's or anti-glomerular basement membrane (GBM) disease is classically characterized by the presence of circulating autoantibodies directed against the non-collagenous domain of the α3 chain of type IV collagen, targeting glomerular and alveolar basement membranes, and associated with rapidly progressive crescentic glomerulonephritis, with alveolar haemorrhage in over half the patients. However, there are increasing examples of variants or atypical presentations of this disease, and novel therapeutic options have been proposed, which nephrologists should be aware of. The pathophysiology of this condition has been understood through molecular analysis of the antibody-antigen interactions and the use of human leucocyte antigen-transgenic animals, while the association of anti-GBM antibodies with anti-neutrophil cytoplasm antibodies and their combined impact on disease phenotype is increasingly recognized, providing some insights into the basis of glomerular damage and autoimmunity. Read More

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February 2018
3 Reads

Anti-glomerular basement membrane disease: Case series from a tertiary center in North India.

Indian J Nephrol 2017 Mar-Apr;27(2):108-112

Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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. Read More

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March 2017
1 Read

The susceptible HLA class II alleles and their presenting epitope(s) in Goodpasture's disease.

Immunology 2017 08 16;151(4):395-404. Epub 2017 May 16.

Renal Division, Peking University First Hospital, Institute of Nephrology, Peking University, Key Laboratory of Renal Disease, Ministry of Health of China, Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China.

Goodpasture's disease is closely associated with HLA, particularly DRB1*1501. Other susceptible or protective HLA alleles are not clearly elucidated. The presentation models of epitopes by susceptible HLA alleles are also unclear. Read More

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August 2017
15 Reads

The pathogenicity of T cell epitopes on human Goodpasture antigen and its critical amino acid motif.

J Cell Mol Med 2017 09 10;21(9):2117-2128. Epub 2017 Mar 10.

Renal Division, Peking University First Hospital, Beijing, China.

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. Read More

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September 2017
20 Reads

Outcome of Renal Transplant in Recipients With Vasculitis.

Exp Clin Transplant 2017 Feb;15(Suppl 1):93-96

Department of Nephrology, Charles Nicolle Hospital, Tunis, Tunisia.

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. Read More

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February 2017
11 Reads

Iminosugar-based ceramide mimicry for the design of new CERT START domain ligands.

Bioorg Med Chem 2017 03 14;25(6):1984-1989. Epub 2017 Feb 14.

SPCMIB, UMR5068 CNRS-Université Paul Sabatier-Toulouse III, 118 route de Narbonne, Toulouse F-31062, France. Electronic address:

The enigmatical dichotomy between the two CERT/GPBP protein isoforms, their vast panel of biological implications and the scarcity of known antagonist series call for new ligand chemotypes identification. We report the design of iminosugar-based ceramide mimics for the development of new START domain ligands potentially targeting either protein isoforms. Strategic choice of (i) an iminoxylitol core structure and (ii) the positioning of two dodecyl residues led to an extent of protein binding comparable to that of the natural cargo lipid ceramide or the archetypical inhibitor HPA-12. Read More

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March 2017
2 Reads

T cell responses to peptides of Goodpasture autoantigen in patients with anti-glomerular basement membrane disease.

Nephrology (Carlton) 2018 Apr;23(4):345-350

Renal Division, Peking University First Hospital; Institute of Nephrology, Peking University; Key Laboratory of Renal Disease, Ministry of Health of China; Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, China.

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. Read More

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April 2018
13 Reads

[Plasma exchange in nephrology: Indications and technique].

Nephrol Ther 2017 Feb 16;13(1):43-55. Epub 2017 Jan 16.

Service des urgences néphrologiques et transplantation rénale, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France; Université Pierre-et-Marie-Curie, 4, place Jussieu, 75005 Paris, France.

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. Read More

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February 2017
19 Reads

Goodpasture Syndrome Diagnosed One Year And A Half after the Appearance of the First Symptoms (Case Report).

Open Access Maced J Med Sci 2016 Dec 6;4(4):683-687. Epub 2016 Dec 6.

University Clinic of Pulmonology and Allergology, Medical Faculty, Ss Cyril and Methodius University of Skopje, Skopje, Republic of Macedonia.

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. Read More

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December 2016
7 Reads

A Case of Pulmonary-Renal Syndrome Leading to the Diagnosis of Legionnaires' Disease.

Case Rep Nephrol 2016 23;2016:4250819. Epub 2016 Nov 23.

Department of Nephrology, Hippokration Hospital, Aristotle University of Thessaloniki, 49 Konstantinoupoleos Street, 54642 Thessaloniki, Greece.

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. Read More

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November 2016
17 Reads

Goodpasture disease as a consequence of melioidosis.

Intern Med J 2016 Dec;46(12):1446-1449

Department of Anatomical Pathology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.

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. Read More

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December 2016
1 Read

Atypical presentation of anti-GBM nephritis in a 90-year-old patient.

BMJ Case Rep 2016 Nov 29;2016. Epub 2016 Nov 29.

Department of Medicine, Sligo University Hospital, Sligo, Co. Sligo, Ireland.

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. Read More

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November 2016
5 Reads

Simultaneous comprehensive multiplex autoantibody analysis for rapidly progressive glomerulonephritis.

Medicine (Baltimore) 2016 Nov;95(44):e5225

aResearch and Development Department, GA Generic Assays GmbH, Dahlewitz/Berlin, GermanybClinical Immunology, San Carlo Borromeo Hospital, Milan, ItalycInstitute of Biotechnology, Faculty Environment and Natural Sciences, Brandenburg University of Technology Cottbus-Senftenberg, SenftenbergdResearch and Development Department, Medipan GmbH, Dahlewitz/Berlin, GermanyeDepartment of Medicine and Surgery, Università degli Studi di Milano - Bicocca (School of Medicine and Surgery), via Cadore, 48 - 20900 Monza (MB)fMicrobiology Institute, San Carlo Borromeo Hospital, Milan, Italy.

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). Read More

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November 2016
23 Reads

[Pulmonary Manifestations of Vasculitis].

Pneumologie 2016 Nov 9;70(11):747-757. Epub 2016 Nov 9.

Zentrum Innere Medizin, Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover.

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. Read More

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November 2016
2 Reads

Vaccine manufacturing and technology: from biotechnological platforms to syntethic epitopes, current viepoint.

Authors:
G A Ignateva

Patol Fiziol Eksp Ter 2016 Oct-Dec;60(4):143-7

The Purposes: The Purposes: the review take into account short history of vaccination practice and development of vaccine technology.

Methods: In the review we include data from several monographs about manufacturing of vaccines published by authors from such companies as Merck & Co; Sanofi Pasteur; Dynavax Europe/Rhein Biotech GmbH; Latham Biopharm Group; Aridis Pharmaceuticals LLC; Genentech; Amgen; Shamir Biologics LLC; Biopharm Services US; Novartis Pharma AG, аnd several research centers: Laboratory of Bacterial Polysaccharides, Center for Biologics Evaluation and Research; Purdue University, West Lafayette, IN, US; Department of Pharmaceutical Chemistry, Univ. Of Kansas; Max Planck Institute for dynamics of Complex Technical Systems; Fraunhofer USA Center for Molecular Biotechnology; US Dep. Read More

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January 2018
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Frequently relapsing anti-glomerular basement membrane antibody disease with changing clinical phenotype and antibody characteristics over time.

Clin Kidney J 2016 Oct 19;9(5):661-4. Epub 2016 Jun 19.

Division of Nephrology, University of British Columbia, 5th Floor Nephrology, 2775 Laurel Street, Vancouver, BC, Canada V5Z 1M9; BC Provincial Renal Agency, Vancouver, BC, Canada; Centre forHealth Evaluation and Outcome Sciences, St. Paul's Hospital, Vancouver, BC, Canada.

Anti-glomerular basement membrane (GBM) antibody disease is a typically monophasic autoimmune disease with severe pulmonary and renal involvement. We report an atypical case of frequently relapsing anti-GBM antibody disease with both anti-GBM antibody-positive flares with pulmonary and renal involvement, and anti-GBM antibody-negative flares that were pulmonary limited with no histologic renal disease. This is the first report of alternating disease phenotype and anti-GBM antibody status over time. Read More

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October 2016
1 Read