2,737 results match your criteria Goodpasture Syndrome


A Rare Case of Double Antibody-Positive Rapidly Progressive Glomerulonephritis: A Therapeutic Challenge.

J Investig Med High Impact Case Rep 2021 Jan-Dec;9:23247096211013193

Florida State University, Sarasota, FL, USA.

Crescentic glomerulonephritis, also known as rapidly progressive glomerulonephritis, is a syndrome characterized by progressive and rapid deterioration of renal function over the course of weeks to months. Oliguria, hematuria, azotemia, and hypertension are characteristic features of this condition. Crescentic glomerulonephritis is further classified according to the staining pattern on immunofluorescence. Read More

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T cell plasticity in renal autoimmune disease.

Cell Tissue Res 2021 May 3. Epub 2021 May 3.

I. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.

The presence of immune cells is a morphological hallmark of rapidly progressive glomerulonephritis, a disease group that includes anti-glomerular basement membrane glomerulonephritis, lupus nephritis, and anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. The cellular infiltrates include cells from both the innate and the adaptive immune responses. The latter includes CD4 and CD8 T cells. Read More

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Anti-glomerular basement membrane disease mediated by IgG and IgA: a case report.

Ren Fail 2021 Dec;43(1):774-778

Department of Nephrology, China-Japan Friendship Hospital, Beijing, People's Republic of China.

Background: Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune condition responsible for rapidly progressive glomerulonephritis. This disease is usually mediated by IgG autoantibodies against the noncollagenous domain of the α3(IV) collagen chain. In rare cases, IgA or IgM anti-GBM antibodies are involved. Read More

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December 2021

Early onset calciphylaxis following acute kidney injury secondary to anti-glomerular basement membrane antibody disease.

BMJ Case Rep 2021 Apr 7;14(4). Epub 2021 Apr 7.

Nephrology and Hypertension, Albany Medical Center Hospital, Albany, New York, USA.

Calciphylaxis is commonly associated with end-stage renal disease (ESRD) and renal transplant. We present a rare case of early onset calciphylaxis in a patient presenting with acute kidney injury (AKI) secondary to anti-glomerular basement membrane (anti-GBM) antibody disease. A 65-year-old obese Caucasian woman with type 2 diabetes mellitus and hypertension presented with a 1-month history of painless gross haematuria and worsening lower extremity oedema. Read More

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Atypical anti-glomerular basement membrane glomerulonephritis in a patient with metastatic melanoma treated with mitogen-activated protein kinase and immune checkpoint inhibitors: a case report.

J Med Case Rep 2021 Apr 3;15(1):186. Epub 2021 Apr 3.

Division of Nephrology, University of Massachusetts Medical School -Baystate, Springfield, MA, USA.

Background: Immune checkpoint inhibitors and mitogen-activated protein kinase inhibitors have become the standard of care in patients with advanced melanoma bearing V600 mutations. However, little is known about their nephrotoxicity. To date, only two cases of anti-glomerular basement membrane glomerulonephritis after exposure to checkpoint inhibitors have been documented. Read More

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Pathology of COVID-19-associated acute kidney injury.

Clin Kidney J 2021 Mar 24;14(Suppl 1):i30-i39. Epub 2021 Jan 24.

Division of Kidney Diseases and Hypertension, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Northwell Health, New Hyde Park, NY, USA.

Acute kidney injury (AKI) is common among hospitalized patients with coronavirus disease 2019 (COVID-19), with the occurrence of AKI ranging from 0.5% to 80%. An improved knowledge of the pathology of AKI in COVID-19 is crucial to mitigate and manage AKI and to improve the survival of patients who develop AKI during COVID-19. Read More

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Relapse of treated anti-GBM disease following hair dye use.

BMJ Case Rep 2021 Apr 1;14(4). Epub 2021 Apr 1.

Department of Nephrology, NHS Dumfries and Galloway, Dumfries, UK

An 18-year-old woman was treated for acute kidney injury (AKI) secondary to antiglomerular basement membrane (GBM) disease with prednisolone, cyclophosphamide and plasma exchange. She also had epistaxis at initial presentation with no other organ involvement and achieved good recovery of her kidney function. Two weeks after completing induction treatment, she re-presented with further AKI and pulmonary haemorrhage. Read More

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Glomerulonephritis in AKI: From Pathogenesis to Therapeutic Intervention.

Front Med (Lausanne) 2020 2;7:582272. Epub 2021 Mar 2.

Nephrology, Dialysis and Transplantation Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.

Acute kidney injury (AKI) is increasingly emerging as a global emergency. Sepsis, major surgery, and nephrotoxic drugs are the main causes of AKI in hospitalized patients. However, glomerulonephritis accounts for about 10% of AKI episodes in adults, mainly related to rapidly progressive glomerulonephritis resulting from granulomatous polyangiitis (GPA, Wegener granulomatosis), microscopic polyangiitis (MPA), and anti-glomerular basement membrane (GBM) disease. Read More

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SARS-CoV-2 infection and recurrence of anti-glomerular basement disease: a case report.

BMC Nephrol 2021 02 27;22(1):75. Epub 2021 Feb 27.

Department of Internal Medicine 3 (Nephrology and Dialysis), Feldkirch Academic Teaching Hospital, Carinagasse 47, A-6800, Feldkirch, Austria.

Background: Anti-glomerular basement membrane disease (GBM) disease is a rare autoimmune disease causing rapidly progressive glomerulonephritis and pulmonary haemorrhage. Recently, an association between COVID-19 and anti-glomerular basement membrane (anti-GBM) disease has been proposed. We report on a patient with recurrence of anti-GBM disease after SARS-CoV-2 infection. Read More

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February 2021

Pediatric double positive anti-glomerular basement membrane antibody and anti-neutrophil cytoplasmic antibody glomerulonephritis-A case report with review of literature.

J Clin Apher 2021 Feb 25. Epub 2021 Feb 25.

Children's Hospital Colorado, Aurora, Colorado, USA.

Anti-glomerular basement membrane (GBM) disease is a rarely described entity in the pediatric population, especially in those less than 3 years old. Even rarer, is double seropositive disease, consisting of anti-GBM antibody plus anti-neutrophil cytoplasmic antibodies. Both single and double antibody positive diseases are characterized by rapidly progressive glomerulonephritis, often without pulmonary involvement in the pediatric population. Read More

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February 2021

A Fatal Case of Diffuse Alveolar Hemorrhage in the Setting of Systemic Lupus Erythematosus: A Case Report and Review of Noninfectious Causes of Acute Pulmonary Hemorrhage in Adults.

Case Rep Rheumatol 2021 11;2021:6620701. Epub 2021 Feb 11.

Department of Laboratory Medicine & Pathology, University of Minnesota Medical School, 420 Delaware Street SE, Minneapolis, MN 55455, USA.

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease, characterized by autoantibody production and immune complex formation, that has the potential to affect virtually any organ. Pleuropulmonary involvement occurs in 50-70% and commonly manifests as pleuritis and pleural effusion. Diffuse alveolar hemorrhage (DAH) is a rare manifestation of SLE. Read More

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February 2021

Anti-glomerular Basement Membrane Disease with Antiphospholipid Syndrome.

Intern Med 2021 Feb 22. Epub 2021 Feb 22.

Department of Nephrology, Nagasaki University Hospital, Japan.

A 48-year-old woman presented with a fever, microscopic hematuria, proteinuria, and rapid deterioration of the renal function. Pulmonary alveolar hemorrhaging and a high level of anti-glomerular basement membrane (GBM) antibodies (700 IU/mL) were observed. Based on her medical history and positive findings of serum lupus anticoagulant, anti-phospholipid antibody syndrome (APS) was suspected. Read More

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February 2021

Seronegative Goodpasture's syndrome associated with organising pneumonia.

BMJ Case Rep 2021 Feb 9;14(2). Epub 2021 Feb 9.

Internal Medicine, Greenwich Hospital/Yale-New Haven Health, Greenwich, Connecticut, USA.

Goodpasture's syndrome is a rare vasculitis associated with anti-glomerular basement membrane (anti-GBM) autoantibodies that target type IV collagen found in the basement membranes of glomeruli and alveoli. We present a case of a 79-year-old man with seronegative Goodpasture's syndrome with predominant respiratory symptoms and mild acute kidney injury that initially improved. Final diagnosis was made by immunofluorescent staining on open lung biopsy which also revealed concomitant organising pneumonia. Read More

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February 2021

Atypical anti-glomerular basement membrane disease with anti-GBM antibody negativity and ANCA positivity: a case report.

BMC Nephrol 2021 Feb 5;22(1):53. Epub 2021 Feb 5.

Kidney Research Institute, Division of Nephrology, West China Hospital, Sichuan University, Chengdu, 610041, China.

Background: Anti-glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disease that involves the lung and kidneys and leads to rapid glomerulonephritis progression, with or without diffuse alveolar hemorrhage, and even respiratory failure. Classic cases of anti-GBM disease are diagnosed based on the presence of the anti-GBM antibody in serum samples and kidney or lung biopsy tissue samples. However, atypical cases of anti-GBM disease are also seen in clinical practice. Read More

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February 2021

Sequential occurrence of microscopic polyangiitis and anti-glomerular basement membrane disease in a patient with small cell lung cancer: a case report.

J Med Case Rep 2021 Feb 1;15(1):30. Epub 2021 Feb 1.

Department of Respirology, Kitano Hospital, The Tazuke Kofukai Medical Research Institute, 2-4-20 Ohgimachi, Kita-ku, Osaka, 530-8480, Japan.

Background: The association between a preceding malignancy and the onset of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has been reported in several studies. While the co-existence of ANCA and anti-glomerular basement membrane (GBM) antibodies in an individual patient is not a common occurrence, this double-positive disease currently has no optimal treatment method. Herein, we report a case of a double-positive disease involving the sequential development of acute kidney injury (AKI) and diffuse alveolar hemorrhage (DAH) in a patient with small cell lung cancer (SCLC). Read More

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February 2021

Anti-Glomerular Basement Membrane Disease as a Potential Complication of COVID-19: A Case Report and Review of Literature.

Cureus 2020 Dec 15;12(12):e12089. Epub 2020 Dec 15.

Nephrology, Lebanese University Faculty of Medicine, Beirut, LBN.

Since the COVID-19 outbreak has started, many reports showed that COVID-19 does not affect only the respiratory system but can alter multiple organs including kidneys. Anti-glomerular basement membrane disease (anti-GBM) is a systemic disease affecting mainly kidneys and lungs. It can sometimes be triggered by a respiratory infection such as influenza however the mechanism is not clear yet. Read More

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December 2020

Atypical anti-glomerular basement membrane disease complicated by methicillin-susceptible Staphylococcus aureus infection-related rapidly progressive glomerulonephritis: a case report and literature review.

CEN Case Rep 2021 Jan 18. Epub 2021 Jan 18.

Department of Nephrology, Asahikawa Red Cross Hospital, 1jo 1 chome, Akebono, Asahikawa, Hokkaido, Japan.

Atypical anti-glomerular basement membrane (GBM) disease, which is characterized by low levels of or negativity for anti-GBM antibodies in circulation but positivity in the kidney, has been recognized in this decade. However, a therapeutic strategy has not been established to date because its outcome is better than that of classic anti-GBM disease. This case report and literature review highlight atypical anti-GBM disease in infection-related rapidly progressive glomerulonephritis. Read More

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January 2021

The clinical and immunological features of the post-extracorporeal shock wave lithotripsy anti-glomerular basement membrane disease.

Ren Fail 2021 Dec;43(1):149-155

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

Introduction: Extracorporeal shock wave lithotripsy (ESWL) is a noninvasive modality to treat urolithiasis, with complications including tissue damage and hematoma of kidney parenchyma. Anti-glomerular basement membrane (GBM) disease is suggested to be a rare complication of ESWL since it was reported in several cases to occur after ESWL. However, the clinical and immunological features of the ESWL-associated anti-GBM disease have not been fully investigated so far. Read More

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December 2021

Concurrent Anti-Glomerular Basement Antibody Disease and Membranous Nephropathy: A Case Series.

Am J Kidney Dis 2021 Jan 7. Epub 2021 Jan 7.

Division of Nephrology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY. Electronic address:

Rationale & Objective: Anti-glomerular basement membrane (GBM) disease is a rapidly progressive glomerulonephritis which, in some instances, occurs concurrently with other diseases such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Rarely, it also occurs with membranous nephropathy (MN). We report a series of such patients, characterizing their long-term follow up. Read More

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January 2021

Absence of Anti-Glomerular Basement Membrane Antibodies in 200 Patients With Systemic Lupus Erythematosus With or Without Lupus Nephritis: Results of the GOODLUPUS Study.

Front Immunol 2020 14;11:597863. Epub 2020 Dec 14.

Département de médecine interne, Centre Hospitalier Universitaire de Reims, Reims, France.

Introduction: Anti-glomerular basement membrane (GBM) antibodies are pathogenic antibodies first detected in renal-limited anti-GBM disease and in Goodpasture disease, the latter characterized by rapidly progressive crescentic glomerulonephritis combined with intra-alveolar hemorrhage. Studies have suggested that anti-GBM antibody positivity may be of interest in lupus nephritis (LN). Moreover, severe anti-GBM vasculitis cases in patients with systemic lupus erythematosus (SLE) have been described in the literature, but few studies have assessed the incidence of anti-GBM antibodies in SLE patients. Read More

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December 2020

Podocyte-specific knockout of the neonatal Fc receptor (FcRn) results in differential protection depending on the model of glomerulonephritis.

PLoS One 2020 28;15(12):e0230401. Epub 2020 Dec 28.

Division of Renal Disease and Hypertension, University of Colorado School of Medicine, Aurora, CO, United States of America.

Podocytes have been proposed to be antigen presenting cells (APCs). In traditional APCs, the neonatal Fc receptor (FcRn) is required for antigen presentation and global knockout of FcRn protects against glomerulonephritis. Since podocytes express FcRn, we sought to determine whether the absence of podocyte FcRn ameliorates immune-mediated disease. Read More

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January 2021

Hypertensive emergency and seizures in a 30-year-old man with anti-glomerular basement membrane disease.

Clin Case Rep 2020 Dec 20;8(12):3322-3327. Epub 2020 Oct 20.

Garnet Health Medical Center Middletown NY USA.

Anti-glomerular basement membrane disease can rapidly lead to renal failure and blood pressure dysregulation. A rare complication is hypertensive encephalopathy in the form of seizures. Patients who have a negative initial seizure workup should have an MRI. Read More

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December 2020

Clinicopathological characteristics and predictors of poor outcome in anti-glomerular basement membrane disease - a fifteen year single center experience.

Ren Fail 2021 Dec;43(1):79-89

Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.

Introduction: Anti-glomerular basement membrane (anti-GBM) disease is a small vessel vasculitis affecting the renal and lung capillary beds. We aim to study the clinicopathological characteristics and predictors of poor outcome of this disease in our population.

Materials And Methods: This is a 15 year retrospective, single center observational study of Indian cohort. Read More

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December 2021

Educational Case: Necrotizing and Crescentic Glomerulonephritis With Anti-Glomerular Basement Membrane Antibodies (Anti-Gbm).

Acad Pathol 2020 Jan-Dec;7:2374289520975166. Epub 2020 Dec 4.

Department of Pathology, University of Iowa, Iowa City, IA, USA.

http://journals.sagepub.com/doi/10. Read More

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December 2020

The Challenging Management of Cancer: An Immunonephrologist's Perspective.

Kidney Blood Press Res 2021 16;46(1):114-120. Epub 2020 Dec 16.

Nephrology and Dialysis Unit & CMID (Center of Research of Immunopathology and Rare Diseases), Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital of Turin, and Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.

Introduction: Onconephrology is an emerging medical subspecialization that focuses on the numberless interrelations between cancer and kidney diseases. Tumor cells evade immune surveillance through activation of immune checkpoint pathways that suppress antitumor immune responses. By blocking checkpoints, new anticancer agents disrupt immune homeostasis but potentially induce immune-mediated diseases. Read More

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December 2020

An uncommon presentation of a rare disease: A case of anti-glomerular basement membrane disease without renal involvement.

Respir Med Case Rep 2020 7;31:101282. Epub 2020 Nov 7.

Baton Rouge General Internal Medicine Residency Program, USA.

Anti-glomerular basement membrane disease (anti-GBM) is a well-documented, small vessel vasculitis that is classically associated with glomerulonephritis and alveolitis [1]. However, regardless of clinical process, not every patient will present with a constellation of classically associated symptoms. Literature review demonstrates that early anti-GBM disease can present as glomerulonephritis without alveolitis [2,3]. Read More

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November 2020

Antineutrophil Cytoplasmic Antibody in Lupus Nephritis: Correlation with Clinicopathological Characteristics and Disease Activity.

Curr Rheumatol Rev 2020 12 8. Epub 2020 Dec 8.

Rheumatology and Rehabilitation Department, Faculty of Medicine, Zagazig University,. Egypt.

Background: Lupus nephritis (LN) represents 40%-50% of all systemic lupus erythematosus (SLE) patients, and rapidly progressive glomerulonephritis is associated with significant morbidity and mortality. Antineutrophil cytoplasmic antibody (ANCA) might be involved in the pathogenesis of LN.

Objective: We evaluated the role of myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA, and anti-glomerular basement membrane autoantibodies (anti-GBM autoAb) for the diagnosis of LN. Read More

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December 2020

Clusterin Deficiency Predisposes C57BL/6j Mice to Cationic Bovine Serum Albumin-Induced Glomerular Inflammation.

J Inflamm Res 2020 24;13:969-983. Epub 2020 Nov 24.

Department of Urologic Sciences, University of British Columbia, Vancouver, BC V6H 3Z6, Canada.

Background: Membranous nephropathy (MN) is a specific entity of glomerulonephritis, and its glomerular inflammation is characterized by the deposition of immune complexes in the glomerular basement membrane and proteinuria. However, the molecular mechanisms underlying the glomerular inflammation of MN are not fully understood. This study was designed to investigate the role of clusterin (CLU) in the development of MN using a mouse model of cationic bovine serum albumin (cBSA)-induced MN. Read More

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November 2020

Long-term outcomes in kidney transplant recipients with end-stage kidney disease due to anti-glomerular basement membrane disease.

Clin Transplant 2021 Feb 12;35(2):e14179. Epub 2020 Dec 12.

Department of Medicine, University of Wisconsin Hospital & Clinics Madison, Madison, WI, USA.

Anti-glomerular basement membrane (GBM) disease causes rapidly progressive glomerulonephritis and end-stage kidney disease (ESKD). Studies of post-transplant outcomes in patients with ESKD due to anti-GBM disease in the United States are lacking. To better characterize outcomes of transplant recipients with a history of anti-GBM disease, we examined patient survival and graft survival among recipients with anti-GBM disease compared with IgA nephropathy at a single center in the United States. Read More

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February 2021

Idiopathic pulmonary hemosiderosis: A state of the art review.

Authors:
Biplab K Saha

Respir Med 2021 01 17;176:106234. Epub 2020 Nov 17.

Division of Pulmonary and Critical Care Medicine, Ozarks Medical Center, West Plains, MO, USA. Electronic address:

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH). Patients with IPH usually present with hemoptysis, and the diagnosis is often delayed by years. Patients often present with intermittent episodes of hemoptysis interspersed between periods of relative normalcy. Read More

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January 2021