2,807 results match your criteria Goodpasture Syndrome


Clinical spectrum of gross haematuria following SARS-CoV-2 vaccination with mRNA vaccines.

Clin Kidney J 2022 May 21;15(5):961-973. Epub 2021 Dec 21.

Division of Nephrology, University Hospital Zurich, Zurich, Switzerland.

Background: Novel messenger RNA (mRNA)-based vaccines play an important role in current vaccination campaigns against SARS-CoV-2. They are highly efficacious and generally well tolerated. Vaccination in patients with immune-mediated kidney diseases is recommended. Read More

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Anti-glomerular basement membrane disease in a patient with Type 2 diabetic mellitus: Long-term remission of the two after immunomodulatory therapy.

Ther Apher Dial 2022 Apr 27. Epub 2022 Apr 27.

Department of Nephrology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India.

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Predictors of Kidney Outcomes of Anti-Glomerular Basement Membrane Disease in a Large Chinese Cohort.

Am J Nephrol 2022 Apr 22:1-10. Epub 2022 Apr 22.

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

Introduction: Anti-glomerular basement membrane (GBM) disease is a rare but the most aggressive form of glomerulonephritis. To dissect the prognostic factors, we retrospectively analyzed the clinical features of a large cohort and compared the clinical features and prognosis during decades.

Methods: Data on clinical manifestation, treatment, and prognosis were collected. Read More

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Acute glomerulonephritis.

Lancet 2022 04;399(10335):1646-1663

Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA. Electronic address:

Glomerulonephritis is a heterogeneous group of disorders that present with a combination of haematuria, proteinuria, hypertension, and reduction in kidney function to a variable degree. Acute presentation with full blown nephritic syndrome or rapidly progressive glomerulonephritis is uncommon and is mainly restricted to patients with post-infectious glomerulonephritis, anti-neutrophil cytoplasmic antibodies-associated vasculitis, and anti-glomerular basement membrane disease. Most frequently, patients present with asymptomatic haematuria and proteinuria with or without reduced kidney function. Read More

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Epidemiology, Impact, and Management Strategies of Anti-Glomerular Basement Membrane Disease.

Int J Nephrol Renovasc Dis 2022 7;15:129-138. Epub 2022 Apr 7.

Department of Laboratory Medicine and Pathology, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar.

Anti-glomerular basement membrane (anti-GBM) disease is a rare but serious autoimmune disease, which is characterized by the development of pathogenic antibodies to type IV collagen antigens in the glomerular and alveolar basement membranes. This results in rapidly progressive glomerulonephritis (GN), alveolar hemorrhage, or both. A variety of environmental factors can trigger the disease in genetically predisposed patients. Read More

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Acquired aplastic anemia complicated with anti-glomerular basement membrane disease successfully treated with immunosuppressive therapy: a case report.

BMC Nephrol 2022 04 7;23(1):136. Epub 2022 Apr 7.

Kidney Disease and Transplant Center, Shonan Kamakura General Hospital, 1370-1, Okamoto, Kamakura, Kanawaga, 247-8533, Japan.

Background: Aplastic anemia (AA) is a rare but fatal disorder characterized by pancytopenia due to bone marrow hypoplasia. Anti-glomerular basement membrane disease (anti-GBM disease) is an immune complex small-vessel vasculitis that presents as rapidly progressive glomerulonephritis and/or pulmonary hemorrhage. Although both involve autoreactive T cells that are partially triggered by human leukocyte antigen (HLA)-DR15, there have been no reports of their co-existence and the treatment strategy is not well understood. Read More

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Rapidly progressive glomerulonephritis in children.

Pak J Med Sci 2022 Jan;38(2):417-425

Farhana Amanullah, MBBS, DABP, FAAP. Pediatric Nephrologist, The Indus Hospital and Health Network, and the Aga Khan University Hospital, Karachi, Pakistan.

Rapidly progressive glomerulonephritis (RPGN), characterized by a rapid development of nephritis with loss of kidney function in days or weeks, is typically associated histologically, with crescents in most glomeruli; and is a challenging problem, particularly in low resource settings. RPGN is a diagnostic and therapeutic emergency requiring prompt evaluation and treatment to prevent poor outcomes. Histopathologically, RPGN consists of four major categories, anti-glomerular basement membrane (GBM) disease, immune complex mediated, pauci-immune disorders and idiopathic /overlap disorders. Read More

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January 2022

Endopeptidase Cleavage of Anti-Glomerular Basement Membrane Antibodies in Severe Kidney Disease: An Open-Label Phase 2a Study.

J Am Soc Nephrol 2022 04 8;33(4):829-838. Epub 2022 Mar 8.

Department of Nephrology and Department of Health, Medicine and Caring Sciences, Linköping University, Linköping, Sweden

Background: The prognosis for kidney survival is poor in patients presenting with circulating anti-glomerular basement membrane (GBM) antibodies and severe kidney injury. It is unknown if treatment with an endopeptidase that cleaves circulating and kidney bound IgG can alter the prognosis.

Methods: An investigator-driven phase 2a one-arm study (EudraCT 2016-004082-39) was performed in 17 hospitals in five European countries. Read More

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Epigenetic Modulation of Gremlin-1/NOTCH Pathway in Experimental Crescentic Immune-Mediated Glomerulonephritis.

Pharmaceuticals (Basel) 2022 Jan 20;15(2). Epub 2022 Jan 20.

Cellular Biology in Renal Diseases Laboratory, IIS-Fundación Jiménez Díaz, Universidad Autónoma Madrid, 28040 Madrid, Spain.

Crescentic glomerulonephritis is a devastating autoimmune disease that without early and properly treatment may rapidly progress to end-stage renal disease and death. Current immunosuppressive treatment provides limited efficacy and an important burden of adverse events. Epigenetic drugs are a source of novel therapeutic tools. Read More

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January 2022

Possible implication of intermolecular epitope spreading in the production of anti-glomerular basement membrane antibody in anti-neutrophil cytoplasmic antibody-associated vasculitis.

Clin Exp Rheumatol 2022 May 4;40(4):691-704. Epub 2022 Feb 4.

Department of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Japan.

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is sometimes complicated by anti-glomerular basement membrane (GBM) disease. Proteases, including elastase, released from neutrophils activated by ANCA are implicated in the pathogenesis of AAV. Epitopes of anti-GBM antibody exist in the α3-subunit non-collagenous (NC1) domain of collagen type IV [Col (IV)]. Read More

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[Glomerular hematuria: an atypical presentation of anti-glomerular basement membrane nephritis].

Ned Tijdschr Geneeskd 2022 01 6;166. Epub 2022 Jan 6.

Meander Medisch Centrum, afd. Interne Geneeskunde, Amersfoort.

Background: Urologic diseases can cause hematuria, but dysmorphic erythrocytes directs to a glomerular disease. The latter might occur isolated or in the presence of systemic complaints, proteinuria or kidney failure. These factors determine the differential diagnosis that ranges from an innocent IgA nephropathy to a fatal anti-glomerular basement membrane (GBM) nephritis. Read More

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January 2022

ANCA-Associated Vasculitis With Anti-GBM Disease and Two Types of Tumors: A Case Report.

Front Med (Lausanne) 2021 21;8:810680. Epub 2022 Jan 21.

Tongde Hospital of Zhejiang Province, Hangzhou, China.

Introduction: Anti-neutrophil cytoplasm antibody (ANCA)-associated-vasculitis and anti-glomerular basement membrane (GBM) disease are types of autoimmune diseases that are characterized by the presence of circulating autoantibodies. Most patients with these diseases experience sudden onset, rapid progress, and poor prognosis. The purpose of the present article is to report a case of ANCA-associated vasculitis with anti-GBM disease and two types of tumors. Read More

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January 2022

Anti-glomerular Basement Membrane Disease: A Rare Case Report of Changing Clinical Phenotype and Atypicalities.

Saudi J Kidney Dis Transpl 2021 May-Jun;32(3):841-850

Department of Nephrology, Government Kilpauk Medical College and Hospital, Chennai, Tamil Nadu, India.

A man in his late 20s, a smoker, presented with nephrotic-range proteinuria and mild renal failure. He had no macroscopic hematuria or decreased urine output. Kidney biopsy was done which revealed a surprising diagnosis of anti-glomerular basement membrane (anti-GBM) disease. Read More

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February 2022

[Update on etiopathogenesis of small vessel vasculitis].

Z Rheumatol 2022 May 27;81(4):270-279. Epub 2022 Jan 27.

Klinik für Rheumatologie und klinische Immunologie, Universität zu Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Small vessel vasculitis is characterized by a necrotizing inflammation of the vessel wall predominantly with involvement of small intraparenchymal arteries, arterioles, capillaries and venules. Medium-sized and occasionally large vessels can also be involved. Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) are differentiated from immune complex vasculitides based on immunopathological and serological aspects. Read More

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Incidence of anti-glomerular basement membrane disease during the COVID-19 pandemic.

Clin Kidney J 2022 Jan 20;15(1):180-181. Epub 2021 Oct 20.

Department of Pathology, Center for Renal and Urological Pathology, Chennai, India.

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January 2022

Diffuse Alveolar Hemorrhage Associated With Severe Acute Respiratory Syndrome Coronavirus 2.

Cureus 2021 Dec 5;13(12):e20171. Epub 2021 Dec 5.

Division of Infectious Diseases, Department of Medicine, Piedmont Columbus Regional Midtown, Columbus, USA.

Diffuse alveolar hemorrhage (DAH) is a rare syndrome resulting from the accumulation of intra-alveolar red blood cells originating most often from the alveolar capillaries and, less frequently, from precapillary arterioles or postcapillary venules. The causes of DAH can be divided into infectious and noninfectious. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a novel coronavirus that has not been previously identified in humans, and it is responsible for coronavirus disease-19 (COVID-19) infection. Read More

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December 2021

Blocking of inflammatory heparan sulfate domains by specific antibodies is not protective in experimental glomerulonephritis.

PLoS One 2021 23;16(12):e0261722. Epub 2021 Dec 23.

Department of Nephrology, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, The Netherlands.

Glomerulonephritis is an acquired serious glomerular disease, which involves the interplay of many factors such as cytokines, chemokines, inflammatory cells, and heparan sulfate (HS). We previously showed that blocking of inflammatory heparan sulfate domains on cultured glomerular endothelium by specific anti-HS single chain antibodies reduced polymorphonuclear cell (PMN) adhesion and chemokine binding. We hypothesized that injection of anti-HS antibodies in PMN-driven experimental glomerulonephritis should reduce glomerular influx of PMNs and thereby lead to a better renal outcome. Read More

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January 2022

An observed association between lung cancer and the presence of anti-Glomerular Basement Membrane antibodies.

Nephrology (Carlton) 2022 Mar 6;27(3):290-292. Epub 2021 Dec 6.

Department of Medicine, St John of God Subiaco Hospital, Subiaco, Australia.

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Anti-Glomerular Basement Membrane Nephritis Potentially Induced by Nebulized Tobramycin Inhalation.

J Aerosol Med Pulm Drug Deliv 2022 Apr 3;35(2):104-106. Epub 2021 Dec 3.

Department of General Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.

To describe a case of anti-glomerular basement membrane (GBM) nephritis that occurred shortly after initiation of nebulized tobramycin (TOB) therapy using intravenous solution, suggesting an association with the inhalation therapy and the disease onset. With the emergence of antimicrobial resistance, clinical importance of aminoglycosides that usually remain susceptibility against gram-negative organisms is increasingly acknowledged. Despite the growing number of evidence supporting the effectiveness of aminoglycoside inhalation therapy for respiratory tract infections, its clinical application has yet to be widely approved by Japanese health insurance. Read More

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[KDIGO 2021 guideline glomerulonephritis - focus on ANCA-associated vasculitides and anti-glomerular basement membrane glomerulonephritis].

Dtsch Med Wochenschr 2021 11 26;146(23):1552-1558. Epub 2021 Nov 26.

Medizinische Klinik und Poliklinik IV, Nephrologisches Zentrum, LMU Klinikum, Campus Großhadern.

In 2021 new KDIGO (Kidney Disease: Improving Global Outcomes) guidelines for the management of glomerular diseases were published.For ANCA-associated glomerulonephritis the new recommendations comprise a more rapid steroid taper during induction treatment with cyclophosphamide or rituximab, the advice against routine use of plasma exchange, the choice of drug for and duration of maintenance treatment in accordance with predictors of relapse. A kidney transplant should be performed after at least 6 months of remission irrespective of the ANCA titer in ANCA-associated disease, and 6 months after absence of anti-GBM-antibodies in anti-GBM-disease. Read More

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November 2021

Anti-glomerular basement membrane disease in children: a brief overview.

Pediatr Nephrol 2021 Nov 12. Epub 2021 Nov 12.

Department of Paediatric Nephrology, Alder Hey Children's NHS Foundation Trust Hospital, Eaton Road, Liverpool, L12 2AP, UK.

Anti-glomerular basement membrane disease (Anti-GBM), previously known as Goodpasture syndrome, is an extremely rare cause of rapidly progressive glomerulonephritis and chronic kidney disease stage 5 (CKD5) in children. It is associated with acute pulmonary haemorrhage and it has a poor prognosis. It is classified as an autoimmune, small-vessel vasculitis caused by autoantibody formation against the alpha-3 chain in type IV collagen found in the glomerular basement membrane. Read More

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November 2021

Pneumocystis pneumonia secondary to intensive immunosuppression treatment for anti-GBM disease complicated with IgA nephropathy: A case report and literature review.

Medicine (Baltimore) 2021 Nov;100(45):e27728

Department of Nephrology, Tianjin Hospital, Tianjin, China.

Rationale: The estimated incidence of anti-glomerular basement membrane (anti-GBM) disease complicated with immunoglobulin A (IgA) nephropathy is minimal, there have only been 15 cases (including this case) reported in the literature, and only 5 (33.33%) of them showed significant improvement in renal function after treatment. Pneumocystis pneumonia is a severe opportunistic pulmonary infection of pneumocystis jiroveci in immunocompromised patients. Read More

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November 2021

Temporal Clustering of Antiglomerular Basement Membrane Disease in COVID-19 Pandemic: A Case Series.

Int J Nephrol Renovasc Dis 2021 1;14:393-398. Epub 2021 Nov 1.

Department of Nephrology, Government Rajaji Hospital, Madurai Medical College, Madurai, Tamil Nadu, India.

Anti-glomerular basement membrane disease (anti-GBM) affects mainly kidneys and lungs. It requires aggressive immunosuppressive treatment. Since the emergence of the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), there has been an increased number of new cases of anti-GBM disease presenting as rapidly progressive glomerulonephritis (RPGN). Read More

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November 2021

Etanercept-Induced Anti-Glomerular Basement Membrane Disease.

Case Rep Nephrol Dial 2021 Sep-Dec;11(3):292-300. Epub 2021 Sep 21.

Department of Renal Medicine, Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, Fulwood, United Kingdom.

Anti-glomerular basement membrane (anti-GBM) disease is a rare form of small-vessel vasculitis that typically causes rapidly progressive glomerulonephritis with or without alveolar haemorrhage. Previously, there has only been one reported case of tumour necrosis factor-α (TNF-α) antagonist-induced anti-GBM disease. Here, we describe the first reported case of etanercept-induced anti-GBM disease. Read More

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September 2021

A Rare Case of Pulmonary-Renal Syndrome With Triple-Seropositive for Myeloperoxidase-Anti-Neutrophil Cytoplasm Antibody (MPO-ANCA), Proteinase 3 (PR3)-ANCA and Anti-Glomerular Basement Membrane (GBM) Antibodies.

J Med Cases 2021 Oct 29;12(10):405-410. Epub 2021 Sep 29.

Department of Internal Medicine, Hospital de Braga, Braga, Portugal.

Anti-glomerular basement membrane (anti-GBM) disease and anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis are the main causes of pulmonary-renal syndrome (PRS). The concurrence of both ANCA - myeloperoxidase (MPO) and proteinase 3 (PR3) - and anti-GBM antibodies has been described, although positivity for all three antibodies has rarely been reported. The natural history of triple-positive patients as well as the best therapeutic approach remains unknown. Read More

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October 2021

The lectin pathway does not contribute to glomerular injury in the nephrotoxic nephritis model.

Nephrology (Carlton) 2022 Feb 2;27(2):208-214. Epub 2021 Nov 2.

Life Sciences and Medicine, King's College London, London, UK.

Aims: Rapidly progressive crescentic glomerulonephritis occurs in number systemic and primary glomerular diseases, including anti-glomerular basement membrane disease, anti-neutrophil cytoplasmic antibody vasculitis and lupus nephritis. Our understanding of pathogenic mechanisms comes from animal models of disease such as the nephrotoxic nephritis model. The lectin pathway of complement activation has been shown to play a key role in several models of inflammation including renal ischaemia reperfusion. Read More

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February 2022

Macroscopic hematuria, facing an uncommon disease: Answers.

Pediatr Nephrol 2022 Feb 19;37(2):339-343. Epub 2021 Oct 19.

Pediatric Nephrology Department, Ondokuz Mayis University Faculty of Medicine, Kurupelit, Samsun, Turkey.

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February 2022

Anti-glomerular basement membrane disease with intense nephrotic syndrome: a new case report.

Pan Afr Med J 2021 16;39:243. Epub 2021 Aug 16.

Department of Nephrology, Dialysis and Kidney Transplantation, La Rabta Hospital, Tunis, Tunisia.

Anti-glomerular basement membrane (anti-GBM) disease was usually described as a small vessel vasculitis presenting with acute kidney injury, haematuria and non-nephrotic proteinuria. We report a case of anti-GBM disease revealed by an intense nephrotic syndrome. The urinary protein level was 12g/day. Read More

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October 2021

Epidemiology and temporal changes in the prognosis of rapidly progressive glomerulonephritis in Japan: a nationwide 1989-2015 survey.

Clin Exp Nephrol 2022 Mar 13;26(3):234-246. Epub 2021 Oct 13.

Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

Background: Rapidly progressive glomerulonephritis (RPGN) can progress to end-stage kidney disease within a short period. This study is a continuation of the chronological nationwide survey conducted by the Japan-RPGN working group.

Methods: We examined a total of 2793 RPGN cases registered during four periods (1989-1998, 1999-2001, 2002-2008, 2009-2011) plus 1386 cases in 2012-2015. Read More

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A rare case of diffuse crescents in fibrillary glomerulonephritis
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Clin Nephrol 2022 Jan;97(1):53-56

Diffuse crescentic involvement in fibrillary glomerulonephritis (FGN) is very rare. We describe a case of FGN with diffuse crescents in a patient who presented with clinical findings concerning for rapidly progressive kidney failure and pathologic findings suggestive of anti-glomerular basement membrane (GBM) disease. Serologies for anti-neutrophil cytoplasmic antibody (ANCA) and anti-GBM were negative. Read More

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January 2022