2,588 results match your criteria Goodpasture Syndrome


Etiology and outcome of pulmonary renal syndrome: Retrospective study from a tertiary care hospitaln.

J Pak Med Assoc 2019 Apr;69(4):588-591

Aga Khan University, KARACHI, PAKISTAN.

To determine the aetiology, clinical characteristics and outcome of patients admitted with pulmonary renal syndrome (PRS). This retrospective analysis was conducted at Aga Khan University Hospital from 2011 to 2015. A total of 17 adult patients admitted with PRS were included and followed up for a period of one year for the outcome of PRS as recovery, dialysis dependency or death. Read More

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April 2019
2 Reads

Rituximab for Anti-Glomerular Basement Membrane Disease.

Kidney Int Rep 2019 Apr 17;4(4):614-618. Epub 2018 Dec 17.

Department of Medicine, Division of Nephrology, Columbia University College of Physicians and Surgeons, New York, New York, USA.

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https://linkinghub.elsevier.com/retrieve/pii/S24680249183034
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http://dx.doi.org/10.1016/j.ekir.2018.12.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451084PMC
April 2019
2 Reads

Incidence, management, and outcomes of autoimmune nephropathies following alemtuzumab treatment in patients with multiple sclerosis.

Mult Scler 2019 Apr 15:1352458519841829. Epub 2019 Apr 15.

School of Medicine, University of Cambridge, Cambridge, UK.

Background: Autoimmune disorders including nephropathies have been reported more frequently in alemtuzumab-treated multiple sclerosis (MS) patients than in the general population.

Objective: Describe instances of autoimmune nephropathy in alemtuzumab-treated MS patients.

Methods: Cases were identified from safety monitoring within the alemtuzumab relapsing-remitting multiple sclerosis (RRMS) clinical development program (CDP) or post-marketing, or following off-label use. Read More

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http://dx.doi.org/10.1177/1352458519841829DOI Listing
April 2019
2 Reads

Chemokine Receptor 8 Can Distinguish Antineutrophil Cytoplasmic Antibody-Associated Vasculitis From Infectious Complications.

Kidney Int Rep 2019 Mar 10;4(3):447-454. Epub 2018 Nov 10.

Division of Nephrology, Japan Community Health Care Organization Sendai Hospital, Sendai, Miyagi, Japan.

Introduction: Diagnosing vasculitis is frequently difficult because its clinical symptoms are similar to those of common infectious diseases and other inflammatory disorders. This study focused on chemokine receptor 8 (CCR8) in peripheral blood mononuclear cells to find a new biomarker that distinguishes vasculitis from infectious complications.

Methods: A cross-sectional study was conducted among 113 patients with systemic vasculitis who were referred to Japan Health Care Organization Sendai Hospital from 2014 to 2016, including those with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, anti-glomerular basement membrane disease, lupus nephritis, and Henoch-Schonlein purpura. Read More

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http://dx.doi.org/10.1016/j.ekir.2018.11.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6409406PMC
March 2019
1 Read

Role of CD8+ T cells in crescentic glomerulonephritis.

Nephrol Dial Transplant 2019 Mar 16. Epub 2019 Mar 16.

Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Crescentic glomerulonephritis (cGN) comprises three main types according to the pathogenesis and immunofluorescence patterns: anti-glomerular basement membrane antibody cGN, vasculitis-associated cGN and post-infectious immune complex cGN. In this brief review of the immune-pathogenesis of cGN, the focus is mainly on the role of CD8+ T cells in the progression of cGN. Under control conditions, Bowman's capsule (BC) provides a protected immunological niche by preventing access of cytotoxic CD8+ T cells to Bowman's space and thereby podocytes. Read More

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http://dx.doi.org/10.1093/ndt/gfz043DOI Listing
March 2019
2 Reads

[Rapid progressive glomerulonephritis].

Authors:
Marion Haubitz

Internist (Berl) 2019 Mar 13. Epub 2019 Mar 13.

Medizinische Klinik III - Nieren- und Hochdruckerkrankungen, Klinikum Fulda gAG, Universitätsmedizin Marburg - Campus Fulda, Pacelliallee 4, 36043, Fulda, Deutschland.

The rapid progressive glomerulonephritis is an emergency case. Renal function is rapidly lost within weeks or a few months (rarely within days) due to necrotizing extracapillary proliferative crescentic glomerulonephritis. Early diagnosis and treatment improve prognosis, as the best prognostic marker is creatinine when treatment is initiated. Read More

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http://link.springer.com/10.1007/s00108-019-0575-x
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http://dx.doi.org/10.1007/s00108-019-0575-xDOI Listing
March 2019
5 Reads

End-stage renal disease secondary to anti-glomerular basement membrane disease in a child with common variable immunodeficiency.

Clin Nephrol Case Stud 2019 1;7:1-6. Epub 2019 Feb 1.

Division of Pediatric Nephrology, Department of Pediatrics.

Background: Anti-glomerular basement membrane (GBM) disease is caused by autoantibodies against the α3-chain of type IV collagen in the GBM. Common variable immunodeficiency (CVID) is a primary immunodeficiency manifested by hypogammaglobulinemia, inability to make functional antibody, and recurrent infections. This report extends the phenotype of CVID-associated autoimmune diseases to include anti-GBM disease. Read More

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http://dx.doi.org/10.5414/CNCS109510DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374989PMC
February 2019
2 Reads

Epitope recognized by anti-glomerular basement membrane (GBM) antibody in a patient with repeated relapse of anti-GBM disease.

Exp Mol Pathol 2019 04 25;107:165-170. Epub 2019 Feb 25.

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

The major epitopes recognized by autoantibodies in anti-glomerular basement membrane (GBM) disease are found in the α3-subunit non-collagenous domain of type IV collagen [α3(IV)NC1], which is present in the glomerular and alveolar basement membranes. These epitopes are structurally cryptic, owing to the hexamer formation of the non-collagenous domain of α3, α4, and α5 subunits and are expressed by the dissociation of the hexamer. Anti-GBM disease usually manifests as a single attack (SA), and we rarely see patients who repeatedly relapse. Read More

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http://dx.doi.org/10.1016/j.yexmp.2019.02.005DOI Listing
April 2019
1 Read

Dual anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody-positive crescent glomerulonephritis in a patient with monoclonal gammopathy of undetermined significance: A case report.

Medicine (Baltimore) 2019 Feb;98(7):e14557

Department of Nephrology.

Rationale: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) antibody disease are both rare autoimmune diseases. Monoclonal gammopathy of undetermined significance (MGUS) is one of the most common causes of plasma cell dyscrasias (PCD). The three entities can cause renal lesions via different mechanisms and, however, they have not been reported in a single patient with renal lesion. Read More

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http://dx.doi.org/10.1097/MD.0000000000014557DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6408057PMC
February 2019
1 Read

A case of aggressive atypical anti-GBM disease complicated by CMV pneumonitis.

BMC Nephrol 2019 Jan 31;20(1):29. Epub 2019 Jan 31.

Department of Pathology and Laboratory Medicine, Cumming School of Medicine, University of Calgary, Foothills Medical Center, 1403 29 St NW, Calgary, AB, T2N 2T9, Canada.

Background: Anti-glomerular basement membrane (anti-GBM) disease is characterized by circulating IgG glomerular basement membrane antibodies and is clinically expressed as a rapidly progressive crescentic glomerulonephritis (GN), with 30-60% of patients also developing pulmonary hemorrhage. Classically, the renal biopsy shows glomerular crescent formation, bright linear staining of glomerular basement membranes (GBM) for IgG on direct immunofluorescence (IF), and the serologic presence of circulating anti-GBM antibodies. Recently, patients with linear IgG IF staining, undetectable circulating anti-GBM antibodies and glomerular changes atypical for anti-GBM disease have been described as "atypical anti-GBM disease", with a distinctly more benign clinical course than typical anti-GBM disease. Read More

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http://dx.doi.org/10.1186/s12882-019-1227-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357502PMC
January 2019
5 Reads

Atypical Goodpasture's disease: a clinical case report and literature review.

Ter Arkh 2018 Jun;90(6):130-136

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

Goodpasture's disease (anti-GBM disease) is a rare small vessels vasculitis characterized by the presence of autoantibodies directed against the glomerular basement membrane (GBM) and alveolar basement membrane. Common feature of anti-GBM disease is a combination of rapidly progressive glomerulonephritis and alveolar hemorrhage (pulmonary-renal syndrome). We present a case of atypical disease course in a young male patient who developed alveolar hemorrhage without renal failure. Read More

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http://dx.doi.org/10.26442/terarkh2018906130-136DOI Listing
June 2018
2 Reads

Pulmonary vasculitis: diagnosis and treatment.

Ter Arkh 2018 Apr;90(3):99-106

Pirogov Russian National Research Medical University, Moscow, Russia.

Pulmonary vasculitis is a group of rapidly progressing severe diseases characterized by vascular inflammation, destruction and necrosis of the pulmonary tissue. The pathological process in the lungs varies from diffuse alveolar hemorrhage to inflammation of the parenchyma, pleural effusion, thrombotic and thromboembolic complications. Depending on the size of the affected vessels, vasculites are divided into vasculites of large, medium and small vessels. Read More

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http://dx.doi.org/10.26442/terarkh201890399-106DOI Listing
April 2018
19 Reads

Androgen deprivation therapy for prostate cancer and the risk of autoimmune diseases.

Prostate Cancer Prostatic Dis 2019 Jan 28. Epub 2019 Jan 28.

Graduate Institute of Life Sciences, National Defense Medical Center, Taipei, Taiwan.

Background: Androgen deprivation therapy (ADT) has been a mainstay of treatment for advanced prostate cancer (PCa), but limited studies have been performed to investigate the association between ADT and autoimmune diseases.

Methods: We conducted a population-based nationwide cohort study of 17,168 patients newly diagnosed with PCa between 1996 and 2013 using the National Health Insurance Research Database (NHIRD) of Taiwan. Cox proportional hazards models with 1:1 propensity score-matched analysis were used to investigate the association between ADT use and the risk of autoimmune diseases. Read More

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http://www.nature.com/articles/s41391-019-0130-9
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http://dx.doi.org/10.1038/s41391-019-0130-9DOI Listing
January 2019
19 Reads

Development of anti-glomerular basement membrane glomerulonephritis during the course of IgA nephropathy: a case report.

BMC Nephrol 2019 Jan 25;20(1):25. Epub 2019 Jan 25.

Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan.

Background: Anti-glomerular basement membrane (GBM) glomerulonephritis does not usually coexist with another glomerulonephritis such as IgA nephropathy. We present a rare case having a combination of these two diseases, and furthermore, histological evaluation could be performed before and after the development of anti-GBM glomerulonephritis over a period of only10 months.

Case Presentation: A 66-year-old woman was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Read More

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http://dx.doi.org/10.1186/s12882-019-1207-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6347754PMC
January 2019
8 Reads

Role of regulatory T cells in experimental autoimmune glomerulonephritis.

Am J Physiol Renal Physiol 2019 Mar 16;316(3):F572-F581. Epub 2019 Jan 16.

Institute of Immunology, University Medical Center Hamburg-Eppendorf , Hamburg , Germany.

Anti-glomerular basement membrane (anti-GBM) disease is characterized by antibodies and T cells directed against the Goodpasture antigen, the noncollagenous domain of the α3-chain of type IV collagen [α3(IV)NC1] of the GBM. Consequences are the deposition of autoantibodies along the GBM and the development of crescentic glomerulonephritis (GN) with rapid loss of renal function. Forkhead box protein P3 (Foxp3) regulatory T (Treg) cells are crucial for the maintenance of peripheral tolerance to self-antigens and the prevention of immunopathology. Read More

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http://dx.doi.org/10.1152/ajprenal.00558.2018DOI Listing
March 2019
4 Reads

Recurrence of Goodpasture syndrome without circulating anti-glomerular basement membrane antibodies after kidney transplant, a case report.

BMC Nephrol 2019 Jan 8;20(1). Epub 2019 Jan 8.

Department of Nephrology, CHU Rennes, Rennes, France.

Background: Goodpasture Syndrome (GS) is an autoimmune disease caused by the development of auto-antibodies against the Glomerular Basement Membrane (GBM). Linear deposit of immunoglobulins G on the GBM detected by immunofluorescence analysis of renal biopsies is a GS pathognomonic finding. GS is commonly monophasic and its incidence is 1. Read More

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https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882
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http://dx.doi.org/10.1186/s12882-018-1197-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323659PMC
January 2019
11 Reads

Acute renal failure in a patient with PR3-ANCA and monoclonal immunoglobulin deposition disease: Case report.

Medicine (Baltimore) 2018 Dec;97(52):e13799

Institute of Health Policy, Management, and Evaluation, University of Toronto, Canada.

Rationale: Acute renal failure developing over a short period of time with evidence of glomerular disease by urine sediment microscopy characterizes the clinical syndrome of rapidly progressive glomerulonephritis (RPGN), of which the most common causes are ANCA-associated glomerulonephritis (GN), immune-complex mediated GN and anti-GBM disease.

Patient Concerns: This was a middle-aged gentleman who presented with acute renal failure and a positive PR3-ANCA.

Diagnosis: Renal biopsy showed an unusual combination of PR3-ANCA GN with focal crescents, monoclonal immunoglobulin deposition disease (MIDD) and mesangial IgA deposition on renal biopsy. Read More

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http://Insights.ovid.com/crossref?an=00005792-201812280-0004
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http://dx.doi.org/10.1097/MD.0000000000013799DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314724PMC
December 2018
5 Reads

Cutaneous vasculitis preceding the onset of anti-GBM disease (Goodpasture syndrome).

Authors:
Nicolas Kluger

Presse Med 2019 Jan 10;48(1 Pt 1):79-80. Epub 2018 Dec 10.

University of Helsinki, Helsinki University Central Hospital, Departments of dermatology, allergology and venereology, Meilahdentie 2, PO Box 160, 00029 Helsinki, Finland. Electronic address:

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http://dx.doi.org/10.1016/j.lpm.2018.11.001DOI Listing
January 2019
1 Read

Activation of fibroblastic reticular cells in kidney lymph node during crescentic glomerulonephritis.

Kidney Int 2019 Feb 3;95(2):310-320. Epub 2018 Dec 3.

Transplantation Research Center, Brigham and Women's Hospital, Boston, Massachusetts, USA; Division of Renal Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA. Electronic address:

Crescentic glomerulonephritis is an inflammatory condition characterized by rapid deterioration of kidney function. Previous studies of crescentic glomerulonephritis have focused on immune activation in the kidney. However, the role of fibroblastic reticular cells, which reside in the stromal compartment of the kidney lymph node, has not been studied in this condition. Read More

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http://dx.doi.org/10.1016/j.kint.2018.08.040DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6342621PMC
February 2019
8 Reads

Acute Thrombotic Microangiopathy and Cortical Necrosis Following Administration of Alemtuzumab: A Case Report.

Am J Kidney Dis 2019 May 7;73(5):615-619. Epub 2018 Dec 7.

Department of Internal Medicine, Wake Forest School of Medicine, Winston-Salem, NC. Electronic address:

Alemtuzumab, a humanized monoclonal antibody that targets CD52 antigens on lymphocytes and monocytes, has shown efficacy in preventing relapse in relapsing-remitting multiple sclerosis. Despite known severe (yet rare) renal side effects such as anti-glomerular basement membrane disease and membranous glomerulopathy, to our knowledge, alemtuzumab has never been documented to cause drug-induced thrombotic microangiopathy. We describe a 39-year-old woman with relapsing-remitting multiple sclerosis who developed acute kidney injury requiring renal replacement therapy after 1 dose of alemtuzumab, as well as microangiopathic hemolytic anemia and thrombocytopenia. Read More

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http://dx.doi.org/10.1053/j.ajkd.2018.09.013DOI Listing
May 2019
17 Reads

Investigations in systemic vasculitis. The role of the laboratory.

Best Pract Res Clin Rheumatol 2018 02 16;32(1):52-62. Epub 2018 Aug 16.

Department of Microbiology and Immunology, KU Leuven, and Department of Laboratory Medicine, University Hospitals Leuven, Leuven, Belgium. Electronic address:

The diagnosis of systemic vasculitis is challenging. Laboratory testing may provide useful information. Routine laboratory tests include erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), blood count, serum creatinine, urinalysis, specific autoantibodies, complement, immunoglobulin, cryoglobulin, and Hepatitis B and C serology. Read More

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http://dx.doi.org/10.1016/j.berh.2018.07.005DOI Listing
February 2018
2 Reads

Extracellular traps in kidney disease.

Kidney Int 2018 12;94(6):1087-1098

Division of Nephrology, Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, LMU München, Germany. Electronic address:

During the past decade the formation of neutrophil extracellular traps (NETs) has been recognized as a unique modality of pathogen fixation (sticky extracellular chromatin) and pathogen killing (cytotoxic histones and proteases) during host defense, as well as collateral tissue damage. Numerous other triggers induce NET formation in multiple forms of sterile inflammation, including thrombosis, gout, obstruction of draining ducts, and trauma. Whether neutrophils always die along with NET release, and if they do die, how, remains under study and is most likely context dependent. Read More

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http://dx.doi.org/10.1016/j.kint.2018.08.035DOI Listing
December 2018
16 Reads

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

Clin Nephrol 2019 Feb;91(2):101-106

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 13 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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http://dx.doi.org/10.5414/CN109584DOI Listing
February 2019
7 Reads

Pulmonary vasculitis.

J Thorac Dis 2018 Sep;10(9):5560-5575

Interdisciplinary Research Group in Pneumology, Institute of Sanitary Research of Santiago de Compostela (IDIS), Santiago de Compostela, Spain.

Systemic vasculitides frequently affect the pulmonary vasculature. As the signs and symptoms of pulmonary vasculitis are variable and nonspecific, diagnosis and treatment represent a real challenge. Vasculitides should be given consideration, as these diseases present severe manifestations of rapidly progressing pulmonary disease. Read More

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http://dx.doi.org/10.21037/jtd.2018.08.117DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196209PMC
September 2018
6 Reads

Treatment of Pulmonary Vasculitis.

Semin Respir Crit Care Med 2018 08 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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http://dx.doi.org/10.1055/s-0038-1669414DOI Listing
August 2018
3 Reads

Antiglomerular Basement Membrane Disease.

Semin Respir Crit Care Med 2018 08 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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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1669413
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http://dx.doi.org/10.1055/s-0038-1669413DOI Listing
August 2018
14 Reads

Alveolar Hemorrhage in Vasculitis (Primary and Secondary).

Semin Respir Crit Care Med 2018 08 7;39(4):482-493. Epub 2018 Nov 7.

Department of Respiratory Medicine, Hospices Civils de Lyon, National Reference Center for Rare Pulmonary Diseases, Lyon, France.

Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Read More

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http://dx.doi.org/10.1055/s-0038-1668533DOI Listing
August 2018
24 Reads

Anti-glomerular basement membrane disease: an update on subgroups, pathogenesis and therapies.

Nephrol Dial Transplant 2018 Oct 29. Epub 2018 Oct 29.

Lund University, Skane University Hospital, Department of Clinical Sciences Lund, Nephrology, Lund, Sweden.

Most patients with anti-glomerular basement membrane (anti-GBM) disease present with rapidly progressive glomerulonephritis with or without pulmonary haemorrhage; however, there are several variants and vigilance is necessary to make a correct diagnosis. Such variants include overlap with anti-neutrophil cytoplasm antibodies-associated vasculitis and membranous nephropathy as well as anti-GBM occurring de novo after renal transplantation. Moreover, patients can present with isolated pulmonary haemorrhage as well as with negative tests for circulating anti-GBM. Read More

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http://dx.doi.org/10.1093/ndt/gfy327DOI Listing
October 2018
16 Reads

Membranous nephropathy followed by anti-glomerular basement disease: A case report and review of clinical presentation and treatment.

SAGE Open Med Case Rep 2018 19;6:2050313X18807621. Epub 2018 Oct 19.

Department of Nephrology, Heidelberg University Hospital, Heidelberg, Germany.

Membranous nephropathy is a common cause of nephrotic syndrome in adults and can be primary or secondary through autoimmune disease, medication, infection, or malignancy. Rapidly progressive glomerulonephritis with crescent formation is rare in patients with membranous nephropathy. Thus, in cases with rapid decline in renal function, after excluding complications such as malignant hypertension, acute hypersensitivity interstitial nephritis, and bilateral renal vein thrombosis, the simultaneous occurrence of a superimposed glomerulonephritis should be considered. Read More

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http://journals.sagepub.com/doi/10.1177/2050313X18807621
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http://dx.doi.org/10.1177/2050313X18807621DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196619PMC
October 2018
13 Reads

Falsely positive anti-glomerular basement membrane antibodies in a patient with hantavirus induced acute kidney injury - a case report.

BMC Nephrol 2018 Oct 22;19(1):286. Epub 2018 Oct 22.

Ziekenhuisgroep Twente, Department of Nephrology, Almelo, The Netherlands.

Background: Hantavirus infection is an uncommon cause of acute renal failure with massive proteinuria. Serology tests to support a presumptive diagnosis usually take a few days. During the initial work-up, autoimmune causes including anti-glomerular basement membrane (GBM) glomerulonephritis need to be excluded, because these require urgent therapy. Read More

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https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882
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http://dx.doi.org/10.1186/s12882-018-1082-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6198376PMC
October 2018
20 Reads

Properdin binds independent of complement activation in an in vivo model of anti-glomerular basement membrane disease.

Kidney Int 2018 12 12;94(6):1141-1150. Epub 2018 Oct 12.

Leiden University Medical Center, Department of Nephrology, Leiden, The Netherlands. Electronic address:

Properdin is the only known positive regulator of complement activation by stabilizing the alternative pathway convertase through C3 binding, thus prolonging its half-life. Recent in vitro studies suggest that properdin may act as a specific pattern recognition molecule. To better understand the role of properdin in vivo, we used an experimental model of acute anti-glomerular basement membrane disease with wild-type, C3- and properdin knockout mice. Read More

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http://dx.doi.org/10.1016/j.kint.2018.06.030DOI Listing
December 2018
8 Reads

Clinicopathological features and outcome of antibody-negative anti-glomerular basement membrane disease.

J Clin Pathol 2019 Jan 12;72(1):31-37. Epub 2018 Oct 12.

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China

Aim: To explore the clinicopathological characteristics of patients with anti-GBM antibody-negative anti-GBM disease.

Methods: The clinical and renal pathological findings were retrospectively studied in 19 patients. All patients met the following inclusion criteria: linear GBM IgG deposition on immunofluorescence(IF); and lack of serum anti-GBM antibodies by ELISA and indirect immunofluorescence assay. Read More

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http://jcp.bmj.com/lookup/doi/10.1136/jclinpath-2018-205278
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http://dx.doi.org/10.1136/jclinpath-2018-205278DOI Listing
January 2019
20 Reads
2.920 Impact Factor

Peroxidasin-a Novel Autoantigen in Anti-GBM Disease?

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

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

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http://dx.doi.org/10.1681/ASN.2018090946DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218874PMC
November 2018
7 Reads

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

Ann Hematol 2019 Apr 9;98(4):1045-1046. Epub 2018 Oct 9.

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

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http://dx.doi.org/10.1007/s00277-018-3513-4DOI Listing
April 2019
3 Reads

Successful treatment of steroid-refractory double-positive ANCA and anti-GBM disease with a combination of plasma exchange and immunosuppression: A case report and literature review.

Respir Med Case Rep 2018 1;25:242-246. Epub 2018 Oct 1.

Division of Neurology, Respirology, Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Kiyotake, Miyazaki, 889-1692, Japan.

The concurrence of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and anti-glomerular basement membrane (GBM) disease, known as double-positive disease, is rare, but it occurs at a much higher frequency than expected by chance. Double-positive disease has an aggressive clinical course, with no optimal treatment strategy. Here we describe a patient with steroid-refractory double-positive disease who was treated successfully with the addition of plasma exchange (PE) and cyclophosphamide (CPA). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22130071183027
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http://dx.doi.org/10.1016/j.rmcr.2018.09.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174835PMC
October 2018
15 Reads

Inhibitory Anti-Peroxidasin Antibodies in Pulmonary-Renal Syndromes.

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

Department of Nephrology and Hypertension,

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

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http://www.jasn.org/lookup/doi/10.1681/ASN.2018050519
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http://dx.doi.org/10.1681/ASN.2018050519DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218858PMC
November 2018
19 Reads

Glomerulonephritis in Animal Models and Human Medicine: Discovery, Pathogenesis, and Diagnostics.

Toxicol Pathol 2018 12 3;46(8):898-903. Epub 2018 Oct 3.

2 University of North Carolina, Chapel Hill, North Carolina, USA.

Glomerulonephritis (GN) is inflammation of glomeruli. The four major categories that cause human GN are mediated by immunoglobulin or complement or both, and they include (1) immune complex-mediated GN, (2) anti-glomerular basement membrane-mediated GN, (3) antineutrophil cytoplasmic autoantibody-mediated GN, and (4) complement factor 3 glomerulopathy mediated by complement dysregulation. Initiating processes include infection, autoimmunity, exogenous antigens, and neoplasia. Read More

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http://dx.doi.org/10.1177/0192623318800714DOI Listing
December 2018
19 Reads

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

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

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

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http://dx.doi.org/10.1111/trf.14765DOI Listing
October 2018
7 Reads

Anti-Glomerular Basement Membrane Disease.

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

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

Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune small vessel vasculitis characterized by autoreactivity to antigens in type IV collagen chains expressed in glomerular and alveolar basement membrane. The detection of circulating anti-GBM antibodies, which are shown to be directly pathogenic, is central to disease diagnosis. Clinically, anti-GBM disease usually presents with rapidly progressive glomerulonephritis with or without alveolar hemorrhage. Read More

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http://dx.doi.org/10.1016/j.rdc.2018.06.011DOI Listing
November 2018
6 Reads

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

Hemodial Int 2019 Apr 25;23(2):E36-E39. Epub 2018 Sep 25.

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

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

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http://dx.doi.org/10.1111/hdi.12671DOI Listing
April 2019
6 Reads

Mid-ventricle Takotsubo in a Patient with Goodpasture Syndrome.

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

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

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

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http://dx.doi.org/10.7759/cureus.2990DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6141219PMC
July 2018
4 Reads

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

BMC Nephrol 2018 09 20;19(1):241. Epub 2018 Sep 20.

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

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

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http://dx.doi.org/10.1186/s12882-018-1038-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6149204PMC
September 2018
3 Reads

Crescentic glomerulonephritis with anti-GBM antibody but no glomerular deposition.

BMC Nephrol 2018 09 12;19(1):228. Epub 2018 Sep 12.

UCL Centre for Nephrology, Royal Free Hospital, London, NW3 2PF, UK.

Background: Anti-glomerular basement membrane (GBM) antibodies are highly specific for Goodpasture's or anti-GBM disease, in which they are generally directed against the non-collagenous (NC1) domain of the alpha 3 chain of type IV collagen(α3(IV)), and less commonly, toward the α 4(IV) or α 5(IV) chains, which form a triple helical structure in GBM and alveolar basement membrane (ABM). Alterations in the hexameric structure of the NC1 (α3 (IV)), allows novel epitopes to be exposed and an immune response to develop, with subsequent linear antibody deposition along the GBM, leading to a crescentic glomerulonephritis. Positive anti-GBM antibodies are assumed to be pathogenic and capable of binding GBM in vivo, especially in the context of rapidly progressive glomerulonephritis. Read More

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http://dx.doi.org/10.1186/s12882-018-1027-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6136232PMC
September 2018
3 Reads

Complement your knowledge with a rare cause of pauci-immune glomerulonephritis.

Clin Rheumatol 2018 Nov 10;37(11):3151-3155. Epub 2018 Sep 10.

Division of Rheumatology, Louisiana State University, 1542 Tulane Ave., Box T4M-2, New Orleans, LA, 70112, USA.

RPGN can be subdivided into three categories on an immunopathologic basis: pauci-immune glomerulonephritis (PIGN), anti-glomerular basement membrane glomerulonephritis (anti-GBM disease), or immune complex-mediated glomerulonephritis (GN). PIGN is the most common cause of RPGN (80% of cases). The most common etiology of PIGN is anti-neutrophil cytoplasmic antibody (ANCA)-associated GN, which accounts for up to 90% of PIGN. Read More

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http://dx.doi.org/10.1007/s10067-018-4275-zDOI Listing
November 2018
4 Reads

Primary antiphospholipid syndrome associated Liebman-Sachs endocarditis leading to diffuse alveolar hemorrhage: A case report.

Respir Med Case Rep 2018 24;25:181-183. Epub 2018 Aug 24.

Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville, USA.

A 26 year old female presented for recurrent blood tinged sputum during the previous year with development of frank hemoptysis three days prior to admission. Diffuse alveolar hemorrhage (DAH) was confirmed with serial lavages. The patient had no history of autoimmune disease, vascular thrombosis or pregnancy morbidity including miscarriages or pre-eclampsia. Read More

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http://dx.doi.org/10.1016/j.rmcr.2018.08.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6125762PMC
August 2018
5 Reads

Standardized classification and reporting of glomerulonephritis.

Nephrol Dial Transplant 2019 Feb;34(2):193-199

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

A kidney biopsy is done to determine the etiology of the glomerulonephritis (GN) and the severity of the lesion, to identify whether other lesions, related to or not related to the GN, are present on the kidney biopsy and finally to ascertain the extent of chronicity of the GN. The etiology of GN is based on the classification of GN into five groups: immune complex-mediated GN, antineutrophil cytoplasmic antibody (ANCA)-associated GN, anti-glomerular basement membrane (GBM) GN, monoclonal immunoglobulin-mediated GN and C3 glomerulopathy. Immune complex GN includes multiple specific diseases such as lupus nephritis, IgA nephropathy, infection-related GN and fibrillary GN. Read More

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http://dx.doi.org/10.1093/ndt/gfy220DOI Listing
February 2019
13 Reads