741 results match your criteria Antiglomerular Basement Membrane Disease


Successful pregnancy in a patient with pulmonary renal syndrome double-positive for anti-GBM antibodies and p-ANCA
.

Clin Nephrol 2018 Nov 15. Epub 2018 Nov 15.

Background: Antiglomerular basement membrane (anti-GBM) antibody disease is a rare condition causing pulmonary hemorrhage and necrotizing glomerulonephritis (pulmonary renal syndrome).

Case: We report a 30-year-old woman who presented with life-threatening pulmonary hemorrhage and an active urinary sediment, with normal glomerular filtration rate in the 13th week of pregnancy. Anti-GBM antibodies in serum were negative, but perinuclear antineutrophil cytoplasmatic antibodies (p--ANCA) were detected. Read More

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November 2018
2 Reads

Renal transplantation in patients with Alport syndrome: patient selection, outcomes, and donor evaluation.

Int J Nephrol Renovasc Dis 2018 16;11:267-270. Epub 2018 Oct 16.

Division of Nephrology, Department of Pediatrics, University of Minnesota Medical School, Minneapolis, MN 55454, USA,

Alport syndrome is an inherited disorder of basement membrane collagen IV that frequently results in end-stage renal disease. Patients with Alport syndrome who undergo renal transplantation have generally excellent outcomes. Posttransplant antiglomerular basement membrane nephritis is a rare complication of renal transplantation for Alport syndrome. Read More

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October 2018
4 Reads

Treatment of Pulmonary Vasculitis.

Semin Respir Crit Care Med 2018 Aug 7;39(4):504-510. Epub 2018 Nov 7.

Department of Internal Medicine, Hôpital Cochin, Université Paris, Paris, France.

Pulmonary vasculitis can be heterogeneous and have some pathophysiological, clinical, and biological specificities that might influence treatment choices. Main vasculitides associated with pulmonary vasculitis include antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, immune complex small vessel vasculitis, and antiglomerular basement membrane disease. Patient characteristics and association of lung involvement with other manifestations affecting disease severity may also influence treatment strategy. Read More

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August 2018
1 Read

Antiglomerular Basement Membrane Disease.

Semin Respir Crit Care Med 2018 Aug 7;39(4):494-503. Epub 2018 Nov 7.

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

Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Read More

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August 2018
4 Reads

Histopathological Classification-A Prognostic Tool for Rapidly Progressive Glomerulonephritis.

Medicina (Kaunas) 2018 Apr 17;54(2). Epub 2018 Apr 17.

Centre of Nephrology, Vilnius University Hospital Santaros Klinikos, LT⁻08661 Vilnius, Lithuania.

Recently proposed histopathological classification may predict patient outcome in pauci-immune glomerulonephritis. This study sought to prove that the prognostic effect could be extended to all types of rapidly progressive glomerulonephritis. Retrospective analysis of patients diagnosed with rapidly progressive glomerulonephritis between April 1999 and August 2015 was performed. Read More

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April 2018
5 Reads

Anti-glomerular basement membrane disease due to monoclonal IgG Lambda antibodies: a very rare case of monoclonal gammopathy of renal significance.

Ann Hematol 2018 Oct 9. Epub 2018 Oct 9.

Pathology Department, Facultad de Medicina, Pontificia Universidad Católica, Santiago de Chile, Chile.

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October 2018
1 Read

Antineutrophil cytoplasmic antibody and/or antiglomerular basement membrane antibody associated crescentic glomerulonephritis in combination with IgG4-related tubulointerstitial nephritis.

Clin Exp Rheumatol 2018 Aug 29. Epub 2018 Aug 29.

Division of Nephrology, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.

Objectives: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder and is characterised by elevated serum IgG4 concentrations and dense lymphoplasmacytic infiltrate rich in IgG4+ plasma cells. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-related kidney disease (IgG4-RKD). We report four cases of kidney injury with concurrent IgG4-TIN and crescentic glomerulonephritis confirmed by renal pathology. Read More

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August 2018
2 Reads
2.720 Impact Factor

A curious case of antiglomerular basement membrane antibody disease.

Clin Case Rep 2018 Aug 1;6(8):1647-1648. Epub 2018 Jul 1.

Division of Pathology, Immunology and Laboratory Medicine University of Florida Gainesville FL USA.

Antiglomerular basement membrane (GBM) antibody disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of autoantibodies. It can be both life- and organ-threatening and delayed diagnosis may be detrimental. Alveolar hemorrhage and microscopic hematuria should be excluded in anybody who is suspected of this disease. Read More

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August 2018
7 Reads

Double-positive with positive anti-glomerular basement membrane antibody and ANCA-positive disease in a patient with dermatomyositis.

BMJ Case Rep 2018 Jul 24;2018. Epub 2018 Jul 24.

Nephrology, Johns Hopkins University, Baltimore, Maryland, USA.

Approximately one in four patients (23.3%) with inflammatory myositis including dermatomyositis can require evaluation for acute kidney injury. The main cause of kidney injury is acute tubular necrosis from medications or myoglobinuria, though clinicians should be aware of a wide variety of possible aetiologies. Read More

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July 2018
3 Reads

Atypical Noncrescentic Antiglomerular Basement Membrane Disease With Concurrent Thin Basement Membrane Nephropathy.

Kidney Int Rep 2018 Jul 23;3(4):991-996. Epub 2018 Mar 23.

Department of Pathology, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada.

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July 2018
2 Reads

Immune System Activation in Rheumatic Heart Disease.

J Coll Physicians Surg Pak 2018 Jul;28(7):518-522

Department of Immunology, University of Health Sciences (UHS), Lahore.

Objective: To assess activation of immune system in rheumatic heart disease (RHD) patients in the form of AECA, ACL and anti GBM antibodies.

Study Design: Descriptive, observational study.

Place And Duration Of Study: Department of Immunology, University of Health Sciences (UHS), Lahore, and Outpatient Department, Punjab Institute of Cardiology, from February 2015 to January 2016. Read More

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July 2018
7 Reads

Proliferative glomerulonephritis with linear immunoglobulin deposition: is this atypical antiglomerular basement membrane disease?

BMJ Case Rep 2018 May 2;2018. Epub 2018 May 2.

Pathology Department, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.

The antiglomerular basement membrane (anti-GBM) antibody disease is marked by the presence of specific antibodies against the non-collagenous domain of the type IV collagen's α3 chain. We describe a case of a 24-year-old Caucasian man, who may have had an atypical presentation of anti-GBM (slow progressive renal insufficiency, massive proteinuria and no detectable circulating anti-GBM antibody). The patient was treated with steroids and cyclophosphamide. Read More

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May 2018
5 Reads

Different effects of global osteopontin and macrophage osteopontin in glomerular injury.

Am J Physiol Renal Physiol 2018 Oct 2;315(4):F759-F768. Epub 2018 May 2.

Department of Medicine, Division of Renal Diseases and Hypertension.

Osteopontin (OPN) is a pro-and anti-inflammatory molecule that simultaneously attenuates oxidative stress. Both inflammation and oxidative stress play a role in the pathogenesis of glomerulonephritis and in the progression of kidney injury. Importantly, OPN is highly induced in nephritic kidneys. Read More

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October 2018
3 Reads

[The 461th case: fever, hematuria, and right lumbar pain].

Zhonghua Nei Ke Za Zhi 2018 Mar;57(3):229-232

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

A 56-year-old female was admitted to the Department of Rheumatology, Peking Union Medical College Hospital with complaint of recurrent fever and acute lumbar pain. Fever was complicated with malaise, cough and occasional blood-streaked sputum. Lab tests showed elevated white blood cell count, increased serum creatinine, erythrocyte sedimentation rate and C-reactive protein. Read More

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March 2018
3 Reads

Successful use of rituximab in glomerular basement membrane nephritis associated with HIV interstitial nephritis secondary to Castleman disease
.

Clin Nephrol 2018 Jun;89(6):469-473

We report a case of glomerular basement membrane crescentic glomerulonephritis and multicentric Castleman disease-associated interstitial nephritis in a patient with human immunodeficiency virus (HIV) infection. The patient received corticosteroids, cyclophosphamide, and plasmapheresis, and within 3 weeks, there was worsening thrombocytopenia, anemia, and renal function requiring initiation of hemodialysis. He then received 8 weekly doses of rituximab, and there was steady improvement in renal function, such that he stopped dialysis within 6 weeks and has remained in disease remission at 1-year follow-up. Read More

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June 2018
6 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

CD44 is required for the pathogenesis of experimental crescentic glomerulonephritis and collapsing focal segmental glomerulosclerosis.

Kidney Int 2018 03 21;93(3):626-642. Epub 2017 Dec 21.

Department of Nephrology, RIMLS, RIHS, Radboud University Medical Center, Nijmegen, The Netherlands. Electronic address:

A key feature of glomerular diseases such as crescentic glomerulonephritis and focal segmental glomerulosclerosis is the activation, migration and proliferation of parietal epithelial cells. CD44-positive activated parietal epithelial cells have been identified in proliferative cellular lesions in glomerular disease. However, it remains unknown whether CD44-positive parietal epithelial cells contribute to the pathogenesis of scarring glomerular diseases. Read More

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March 2018
18 Reads
8.560 Impact Factor

Coexistence of Anti-Glomerular Basement Membrane Glomerulonephritis and Membranous Nephropathy in a Female Patient with Preserved Renal Function.

Tohoku J Exp Med 2017 12;243(4):335-341

Department of Nephrology, Nagano Red Cross Hospital.

Renal prognosis for anti-glomerular basement membrane (GBM) glomerulonephritis is poor. The greater the amount of anti-GBM antibody binding the antigen (type IV collagen of the glomerular basement membrane), the greater the number of crescents that develop in glomeruli, resulting in progression of renal impairment. Immunofluorescence staining reveals linear IgG depositions on glomerular capillary walls. Read More

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December 2017
16 Reads

Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury.

Saudi J Kidney Dis Transpl 2017 Nov-Dec;28(6):1397-1403

Department of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.

Antiglomerular basement membrane (anti-GBM) disease is an uncommon autoimmune disease characterized by the presence of IgG autoantibodies targeting the alpha-3 chain of type IV collagen. Some of the atypical forms of the disease have been described. Herein, we describe a case of atypical anti-GBM in a 27-year-old Saudi male who presented with lower limb edema, gross hematuria, elevated serum creatinine concentration, and nephrotic-range proteinuria. Read More

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December 2017
14 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

Discontinuation of Hemodialysis in a Patient with Anti-GBM Disease by the Treatment with Corticosteroids and Plasmapheresis despite Several Predictors for Dialysis-Dependence.

Case Rep Nephrol 2017 11;2017:7143649. Epub 2017 Oct 11.

Department of Internal Medicine, Teikyo University School of Medicine, Itabashi-ku, Tokyo, Japan.

A 26-year-old man highly suspected of having antiglomerular basement membrane (GBM) disease was treated with corticosteroid pulse therapy 9 days after initial infection-like symptoms with high procalcitonin value. The patient required hemodialysis the next day of the treatment due to oliguria. In addition to corticosteroid therapy, plasmapheresis was introduced and the patient could discontinue hemodialysis 43 days after the treatment. Read More

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October 2017
10 Reads

Immunoadsorption in Anti-GBM Glomerulonephritis: Case Report in a Child and Literature Review.

Pediatrics 2017 Nov;140(5)

Department of Pediatric Nephrology, MARHEA - Necker Hospital - APHP, Imagine Institute, Paris Descartes University, Paris, France; and

Antiglomerular basement membrane glomerulonephritis (anti-GBM GN) is a rare autoimmune disease that is characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage. Anti-GBM GN is caused by autoantibodies (classically type G immunoglobulin) directed against the α3 subunit of type IV collagen. Without any appropriate treatment, the disease is generally fulminant, and patient and kidney survival is poor. Read More

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November 2017
7 Reads

ID3 may protect mice from anti‑GBM glomerulonephritis by regulating the differentiation of Th17 and Treg cells.

Mol Med Rep 2017 Dec 4;16(6):9086-9094. Epub 2017 Oct 4.

Division of Nephrology, Department of Internal Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430030, P.R. China.

Anti‑glomerular basement membrane glomerulonephritis (anti‑GBM GN) is an autoimmune disease that leads to severe and rapidly progressive renal injury. Inhibition of DNA‑binding factor 3 (ID3) serves a key role in autoimmune diseases, such as asthma and Sjögren's syndrome, and in experimental allergic encephalitis models. However, the role of ID3 in the progression of anti‑GBM GN remains unknown. Read More

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December 2017
28 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

The Sequential Development of Antiglomerular Basement Membrane Nephritis and Myeloperoxidase-antineutrophil Cytoplasmic Antibody-associated Vasculitis.

Intern Med 2017 Oct 6;56(19):2617-2621. Epub 2017 Sep 6.

Internal Medicine 1, Hamamatsu University School of Medicine, Japan.

A 55-year-old woman presented with deafness, increased levels of myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA), and renal insufficiency with proteinuria and hematuria. Renal biopsy revealed crescentic glomerulonephritis with the linear deposition of immunoglobulin G along the glomerular basement membrane (GBM) and peritubular capillaritis. The anti-GBM antibody levels on admission and 10 days after admission were 11. Read More

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October 2017
7 Reads

Therapeutic Mechanism of Glucocorticoids on Cellular Crescent Formation in Patients With Antiglomerular Basement Membrane Disease.

Am J Med Sci 2017 08 25;354(2):145-151. Epub 2017 Apr 25.

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China. Electronic address:

Background: This study aimed to explore the therapeutic mechanism of glucocorticoids (GCs) in antiglomerular basement membrane disease.

Materials And Methods: Thirty-four patients with biopsy-proven antiglomerular basement membrane nephritis were divided into the following 2 groups: group 1 (patients treated with GCs, n = 22) and group 2 (patients who were not treated with GCs, n = 12). The expression of parietal epithelial cells (PECs), activated PECs and glucocorticoid receptors (GRs) was examined quantitatively and compared between the 2 groups. Read More

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August 2017
17 Reads

Familial antiglomerular basement membrane disease in zero human leukocyte antigen mismatch siblings
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Clin Nephrol 2017 Nov;88(11):277-283

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. Read More

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November 2017
21 Reads

Clinicopathological and long-term prognostic features of membranous nephropathy with crescents: a Japanese single-center experience.

Clin Exp Nephrol 2018 Apr 29;22(2):365-376. Epub 2017 Aug 29.

Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, Akita, 010-8543, Japan.

Background: Three recent studies from the United States and China reported the clinicopathological features and short-term prognosis in patients with membranous nephropathy (MN) and crescents in the absence of secondary MN, anti-glomerular basement membrane (GBM) antibodies, and anti-neutrophil cytoplasmic antibodies (ANCA).

Methods: We compared clinicopathological and prognostic features in 16 MN patients with crescents (crescent group) and 38 MN patients without crescents (control group), in the absence of secondary MN, anti-GBM antibodies, and ANCA. Median follow-up periods in the crescent and control groups were 79 and 50 months, respectively. Read More

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April 2018
5 Reads

Effectiveness of Plasmapheresis in a Patient with Anti-glomerular Basement Membrane Antibody Glomerulonephritis with Advanced Kidney Dysfunction.

Intern Med 2017 Sep 21;56(18):2475-2479. Epub 2017 Aug 21.

Department of Nephrology, Faculty of Medicine, University of Tsukuba, Japan.

Patients with anti-glomerular basement membrane antibody glomerulonephritis (anti-GBM GN) have severe kidney dysfunction, leading to end-stage renal disease. The effect of plasmapheresis and immunosuppressive treatment in patients with severe glomerular changes is controversial. A 62-year-old man was admitted with rapidly progressive glomerulonephritis and diagnosed with anti-GBM GN. Read More

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September 2017
8 Reads

ANCA in anti-GBM disease: moving beyond a one-dimensional clinical phenotype.

Kidney Int 2017 09;92(3):544-546

Trinity Health Kidney Centre, Trinity College Dublin, Dublin, Ireland.

McAdoo et al. propose that patients with both circulating antiglomerular basement membrane antibody and antineutrophil cytoplasm antibody demonstrate a phenotype that lies between that of single-positive antiglomerular basement membrane disease and antineutrophil cytoplasm antibody-associated vasculitis. Specifically, there may be a subset of "double-positives" that have a more favorable response to therapy. Read More

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September 2017
6 Reads

Retinoic acid improves nephrotoxic serum-induced glomerulonephritis through activation of podocyte retinoic acid receptor α.

Kidney Int 2017 12 27;92(6):1444-1457. Epub 2017 Jul 27.

Department of Medicine, Division of Nephrology, Icahn School of Medicine at Mount Sinai, New York, USA; Department of Pharmacological Sciences, Icahn School of Medicine at Mount Sinai, New York, New York, USA; Renal Section, James J Peters VAMC, Bronx, New York, USA. Electronic address:

Proliferation of glomerular epithelial cells, including podocytes, is a key histologic feature of crescentic glomerulonephritis. We previously found that retinoic acid (RA) inhibits proliferation and induces differentiation of podocytes by activating RA receptor-α (RARα) in a murine model of HIV-associated nephropathy. Here, we examined whether RA would similarly protect podocytes against nephrotoxic serum-induced crescentic glomerulonephritis and whether this effect was mediated by podocyte RARα. Read More

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December 2017
12 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

Antiglomerular Basement Membrane Disease in a Pediatric Patient: A Case Report and Review of the Literature.

Case Rep Nephrol 2017 9;2017:1256142. Epub 2017 May 9.

Department of Pediatric Nephrology, University of Illinois College of Medicine at Peoria, Peoria, IL, USA.

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. Read More

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May 2017
15 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

Anti-Glomerular Basement Membrane Disease.

Clin J Am Soc Nephrol 2017 Jul 17;12(7):1162-1172. Epub 2017 May 17.

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

Anti-glomerular basement membrane (anti-GBM) disease is a rare small vessel vasculitis that affects the capillary beds of the kidneys and lungs. It is an archetypic autoimmune disease, caused by the development of directly pathogenic autoantibodies targeting a well characterized autoantigen expressed in the basement membranes of these organs, although the inciting events that induce the autoimmune response are not fully understood. The recent confirmation of spatial and temporal clustering of cases suggests that environmental factors, including infection, may trigger disease in genetically susceptible individuals. Read More

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July 2017
10 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

What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report.

Transplant Proc 2017 May;49(4):913-915

Urology and Renal Transplantation Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Introduction: Collapsing glomerulopathy (CG) is a rare form of glomerular injury. Although commonly associated with human immunodeficiency virus (HIV) infection, it can occur in association with systemic lupus erythematosus (SLE).

Case Report: We present the case of a 50-year-old man, with chronic kidney disease secondary to focal and segmental glomerulosclerosis, who received a cadaveric kidney transplant in 2007. Read More

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May 2017
3 Reads

Pre-existing chronic interstitial pneumonia is a poor prognostic factor of Goodpasture's syndrome: a case report and review of the literature.

J Med Case Rep 2017 Apr 13;11(1):102. Epub 2017 Apr 13.

Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga, 849-8501, Japan.

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. Read More

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April 2017
13 Reads

Clinicopathological characteristics of typical and atypical anti-glomerular basement membrane nephritis.

J Nephrol 2017 Aug 5;30(4):503-509. Epub 2017 Apr 5.

Department of Surgery and Translational Medicine, Pathology, University Milan Bicocca, San Gerardo Hospital, Monza, Italy.

Anti-glomerular basement membrane (GBM) antibody disease is a rare pathological condition that mainly involves renal and/or pulmonary parenchyma. It is characterized by the presence of circulating anti-GBM antibodies accompanied by a linear deposition of immunoglobulins (Ig) detected through immunofluorescence (IF) technique and typical signs and symptoms of organ dysfunction, such as rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage (PH). However, recently atypical forms of anti-GBM disease have been described and the presence of overlapping diseases contributed to make its diagnosis challenging. Read More

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August 2017
12 Reads

Goodpasture's disease with late presentation of renal abnormality and anti-GBM autoantibody.

BMJ Case Rep 2017 Mar 17;2017. Epub 2017 Mar 17.

Royal Bolton Hospital NHS Foundation Trust, Bolton, UK.

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. Read More

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March 2017
10 Reads

Glomerular common gamma chain confers B- and T-cell-independent protection against glomerulonephritis.

Kidney Int 2017 05 19;91(5):1146-1158. Epub 2017 Jan 19.

Unité Mixte de Recherche (UMR) 1155, Institut National de la Santé et de la Recherche Médicale (Inserm), Paris, France; Sorbonne Universités, Université Pierre et Marie Curie Paris 06, Paris, France; Urgences Néphrologiques et Transplantation Rénale, Hôpital Tenon, Assistance Publique - Hôpitaux de Paris, Paris, France; Weill Cornell Medicine, The HRH Prince Alwaleed Bin Talal Bin Abdulaziz Alsaud Institute for Computational Biomedicine, New York, New York, USA. Electronic address:

Crescentic glomerulonephritis is a life-threatening renal disease that has been extensively studied by the experimental anti-glomerular basement membrane glomerulonephritis (anti-GBM-GN) model. Although T cells have a significant role in this model, athymic/nude mice and rats still develop severe renal disease. Here we further explored the contribution of intrinsic renal cells in the development of T-cell-independent GN lesions. Read More

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May 2017
10 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
2 Reads

Novel biomarkers for the assessment of paediatric systemic lupus erythematosus nephritis.

Clin Exp Immunol 2017 04 22;188(1):79-85. Epub 2017 Jan 22.

1st Department of Pediatrics, Aristotle University, 'Hippokration' General Hospital, Thessaloniki, Greece.

The discovery of serum biomarkers specific for paediatric lupus nephritis (pLN) will facilitate the non-invasive diagnosis, follow-up and more appropriate use of treatment. The aim of this study was to explore the role of serum high-mobility group box 1 (HMGB1) protein, antibodies against nucleosomes (anti-NCS), complement factor C1q (anti-C1q) and glomerular basement membrane (anti-GBM) in pLN. Serum samples of 42 patients with paediatric systemic lupus erythematosus (pSLE) (22 with pLN and 20 without renal involvement), 15 patients with other autoimmune nephritis (AN) and 26 healthy controls (HCs) were examined using enzyme-linked immunosorbent assay (ELISA). Read More

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April 2017
2 Reads

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
6 Reads

Diffuse Alveolar Hemorrhage Associated with Edoxaban Therapy.

Case Rep Crit Care 2016 31;2016:7938062. Epub 2016 Oct 31.

Department of Emergency and Critical Care Medicine, Shinshu University School of Medicine, Nagano, Japan.

The main adverse effect of anticoagulant therapy is bleeding, and major bleeding, including intracranial, gastrointestinal, and retroperitoneal bleeding, has been reported as an adverse effect of edoxaban, a direct oral anticoagulant (DOAC). Bleeding during systemic anticoagulation with edoxaban presents a therapeutic conundrum, because there is currently no safe or efficacious reversal agent to stop major bleeding. A 51-year-old woman had multiple traumatic injuries, including lower limb fractures. Read More

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October 2016
6 Reads

Avidity of selected autoantibodies - usefulness of their determination for clinical purposes.

Authors:
L Fialová

Epidemiol Mikrobiol Imunol Fall 2016;65(3):155-163

Autoantibodies directed against various self-antigens comprise a heterogeneous group of immunoglobulins, which differ in their qualitative and quantitative features. An important qualitative characteristic of antibodies is affinity/avidity, which changes in the process of its maturation during the immune response.This study is aimed to summarize the knowledge about avidity of selected autoantibodies in certain autoimmune diseases. Read More

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October 2017
2 Reads

Atypical Antiglomerular Basement Membrane Disease With IgG1-κ Staining.

Kidney Int Rep 2017 Jan 27;2(1):80-83. Epub 2016 Aug 27.

Renal Division, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.

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January 2017
2 Reads

Optimizing the translational value of animal models of glomerulonephritis: insights from recent murine prototypes.

Authors:
Mary H Foster

Am J Physiol Renal Physiol 2016 09 22;311(3):F487-95. Epub 2016 Jun 22.

Department of Medicine, Duke University Medical Center, Durham, North Carolina; and Durham Veterans Affairs Medical Center, Durham, North Carolina

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. Read More

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September 2016

Outcomes of patients with Goodpasture syndrome: A nationwide cohort-based study from the French Society of Hemapheresis.

J Autoimmun 2016 09 4;73:24-9. Epub 2016 Jun 4.

CHU de Toulouse, Département de Néphrologie et Transplantation d'Organes, Centre de Référence des maladies rénales rares, Hôpital Rangueil, Université Paul Sabatier - Toulouse III, Toulouse, France; INSERM U1048 (équipe 12), Institut des Maladies Métaboliques et Cardiovasculaires (I2MC), Toulouse, France. Electronic address:

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. Read More

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September 2016
17 Reads

Factors Associated With Pathogenicity of Anti-Glomerular Basal Membrane Antibodies: A Case Report.

Medicine (Baltimore) 2016 May;95(19):e3654

From the Urgences Néphrologiques et Transplantation Rénale (RO, JP, AH, ER, PG); Service d'Anatomie Pathologique (DB, IB); Service de Néphrologie et Dialyses (KD), Hôpital Tenon; Université Pierre et Marie Curie (DB, IB, AH, ER, PG), (Paris 6), Sorbonne Universités, Paris, France; Department of Nephrology (TH), Clinical Sciences in Lund, Lund University, Lund, Sweden; and Laboratoire Jean Dausset (LabEX Transplantex) et Inserm UMRS 1160 (RT), Hôpital Saint Louis, Paris, France.

Antiglomerular basement membrane (GBM) disease is known as a super-acute proliferative glomerulonephritis caused by auto-antibodies targeting the NC1 domain of the α3 chain of type IV collagen.Here, we describe a case of atypical anti-GBM disease presenting as a dialysis-dependent acute renal failure with unusual mild glomerular involvement. We found that immunoglobulin G (IgG) deposits were restricted to the uncommon IgG2 and IgG4 subclasses, and that blood was positive for anti-GBM antibodies by immunofluorescence, but not by Enzyme Linked Immunosorbent Assay (ELISA). Read More

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May 2016
16 Reads