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https://www.readbyqxmd.com/read/28927772/-antiglomerular-basement-disease-in-children-literature-review-and-therapeutic-options
#1
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/28886586/a-murine-ig-light-chain-transgene-reveals-igkv3-gene-contributions-to-anti-collagen-types-iv-and-ii-specificities
#2
Amy G Clark, Inge M Worni-Schudel, Francesca M Korte, Mary H Foster
A subset of autoimmune diseases result from autoantibodies targeting epitopes on matrix collagen. The most extensively studied are anti-glomerular basement membrane glomerulonephritis (or its systemic counterpart Goodpasture's disease) that destroys kidneys and lungs, and rheumatoid arthritis that leads to disabling arthritis. Autoantibodies in these disorders bind evolutionarily conserved conformational epitopes on the noncollagenous domain 1 (NC1) of the alpha3 chain of type IV [alpha3(IV)NC1] collagen in glomerular and alveolar basement membranes, and on native or citrullinated type II collagen (CII) in joint cartilage, respectively...
September 5, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28863794/immunoadsorption-in-autoimmune-diseases-affecting-the-kidney
#3
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
#4
Andrea Angioi, Wisit Cheungpasitporn, Sanjeev Sethi, An De Vriese, Nicola Lepori, Thomas Schwab, Fernando 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...
August 30, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28796467/anti-glomerular-basement-membrane-antibody-diagnostics-in-a-large-cohort-tertiary-center-should-we-trust-serological-findings
#5
Abdulla Watad, Nicola Luigi Bragazzi, Kassem Sharif, Ora Shovman, Boris Gilburd, Howard Amital, Yehuda Shoenfeld
BACKGROUND: Anti-glomerular basement membrane (GBM) antibody disease, or Goodpasture's disease, is the clinical manifestation of the production of anti-GBM antibodies, which causes rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Anti-GBM antibody detection is mandatory for the diagnosis of Goodpasture's disease either from the serum or kidney biopsy. Renal biopsy is necessary for disease confirmation; however, in cases in which renal biopsy is not possible or is delayed, serum detection of anti-GBM antibody is the only way for diagnosis...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28765179/status-epilepticus-as-the-initial-presentation-of-antibody-negative-goodpasture-s-syndrome
#6
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
#7
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
#8
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/28570922/the-critical-amino-acids-of-a-nephritogenic-epitope-on-human-goodpasture-autoantigen-for-binding-to-hla-drb1-1501
#9
Qiu-Hua Gu, Xiao-Yu Jia, Jian-Nan Li, Fang-Jin Chen, Zhao Cui, Ming-Hui Zhao
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 α3127-148 (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. METHODS: A line of EBV-transformed human B cells homozygous for HLA-DRB1*1501 was used to detect the binding capacity of peptides to HLA-DRB1*1501 using flow cytometry analysis...
May 29, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28550388/antibodies-against-linear-epitopes-on-goodpasture-autoantigen-in-patients-with-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#10
Xiao-Yu Jia, Jun-Tao Yu, Shui-Yi Hu, Jian-Nan Li, Miao Wang, Chen Wang, Min Chen, Zhao Cui, Ming-Hui Zhao
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...
May 26, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28515462/autoimmunity-hla-mediated-protection-in-goodpasture-disease
#11
Ellen F Carney
No abstract text is available yet for this article.
July 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28502986/autoimmunity-hla-mediated-protection-in-goodpasture-disease
#12
Ellen F Carney
No abstract text is available yet for this article.
July 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28467828/dominant-protection-from-hla-linked-autoimmunity-by-antigen-specific-regulatory-t-cells
#13
Joshua D Ooi, Jan Petersen, Yu H Tan, Megan Huynh, Zoe J Willett, Sri H Ramarathinam, Peter J Eggenhuizen, Khai L Loh, Katherine A Watson, Poh Y Gan, Maliha A Alikhan, Nadine L Dudek, Andreas Handel, Billy G Hudson, Lars Fugger, David A Power, Stephen G Holt, P Toby Coates, Jon W Gregersen, Anthony W Purcell, Stephen R Holdsworth, Nicole L La Gruta, Hugh H Reid, Jamie Rossjohn, A Richard Kitching
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 (α3135-145)...
May 11, 2017: Nature
https://www.readbyqxmd.com/read/28459999/diagnostic-and-management-challenges-in-goodpasture-s-anti-glomerular-basement-membrane-disease
#14
Scott R Henderson, Alan D Salama
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...
April 28, 2017: Nephrology, Dialysis, Transplantation
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
#15
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
#16
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/28342268/the-susceptible-hla-class-ii-alleles-and-their-presenting-epitope-s-in-goodpasture-s-disease
#17
Li-Jun Xie, Zhao Cui, Fang-Jin Chen, Zhi-Yong Pei, Shui-Yi Hu, Qiu-Hua Gu, Xiao-Yu Jia, Li Zhu, Xu-Jie Zhou, Hong Zhang, Yun-Hua Liao, Lu-Hua Lai, Billy G Hudson, Ming-Hui Zhao
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. We genotyped 140 Chinese patients and 599 controls for four-digit HLA II genes, and extracted the encoding sequences from the IMGT/HLA database. T-cell epitopes of α3(IV)NC1 were predicted and the structures of DR molecule-peptide-T-cell receptor were constructed. We confirmed DRB1*1501 (OR = 4·6, P = 5·7 × 10(-28) ) to be a risk allele for Goodpasture's disease...
August 2017: Immunology
https://www.readbyqxmd.com/read/28314809/goodpasture-s-disease-with-late-presentation-of-renal-abnormality-and-anti-gbm-autoantibody
#18
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
#19
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...
September 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28260443/outcome-of-renal-transplant-in-recipients-with-vasculitis
#20
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
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