2,662 results match your criteria Goodpasture Syndrome


Cyclophosphamide pulse therapy as treatment for severe interstitial lung diseases.

Sarcoidosis Vasc Diffuse Lung Dis 2019 1;36(2):157-166. Epub 2019 May 1.

Department of Medicine A, Hematology, Oncology and Pneumology, University Hospital Muenster, Muenster, Germany.

Introduction: Besides invasive or non-invasive ventilation, treatment of severe forms of interstitial lung diseases (ILD) includes immunosuppressive medication. In case of refractory organ- or life-threatening courses of disease, cyclophosphamide pulse therapy can serve as a rescue treatment option.

Objectives: To investigate therapeutic and prognostic effects of cyclophosphamide for the treatment of severe forms of ILD on intensive care unit (ICU) we performed this analysis. Read More

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http://dx.doi.org/10.36141/svdld.v36i2.7636DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7247101PMC

Positron emission tomography imaging of renal mitochondria is a powerful tool in the study of acute and progressive kidney disease models.

Kidney Int 2020 Mar 28. Epub 2020 Mar 28.

Nephrology Research Laboratories, Kyowa Kirin Co., Ltd., Shizuoka, Japan.

Mitochondrial dysfunction plays a critical role in the pathogenesis of kidney diseases via ATP depletion and reactive oxygen species overproduction. Nonetheless, few studies have reported the renal mitochondrial status clinical settings, partly due to a paucity of methodologies. Recently, a positron emission tomography probe, F-BCPP-BF, was developed to non-invasively visualize and quantitate the renal mitochondrial status in vivo. Read More

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http://dx.doi.org/10.1016/j.kint.2020.02.024DOI Listing

Heavy chain deposition disease presenting with raised anti-GBM antibody levels; a case report.

BMC Nephrol 2020 May 12;21(1):175. Epub 2020 May 12.

Renal Unit, Royal Berkshire Hospital, London Road, Reading, Berkshire, UK.

Background: Monoclonal immunoglobulin deposition disease (MIDD) is a rare condition accounting for < 1% of histopathological diagnoses made on kidney biopsy. The best outcomes are seen in those diagnosed and treated promptly, but delay to diagnosis is common with the largest series reporting a median time from onset of renal impairment to diagnosis of 12 months. Here, we report a case of the heavy chain subset of MIDD presenting with positive anti-glomerular basement membrane (anti-GBM) antibodies obscuring the true diagnosis. Read More

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http://dx.doi.org/10.1186/s12882-020-01837-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7216388PMC

Atypical Anti-Glomerular Basement Membrane Disease With Diffuse Crescentic Membranoproliferative Glomerulonephritis: Case Report and Review of Literature.

Qatar Med J 2020 5;2020(1):16. Epub 2020 May 5.

Division of Nephrology, Department of Medicine, Hamad Medical Corporation, Doha, Qatar.

Anti-glomerular basement membrane (anti-GBM) disease occurs in fewer than two cases per million population. Patients usually present with features of rapidly progressive glomerulonephritis (RPGN) with or without pulmonary involvement. Anti-GBM disease is classically diagnosed by both demonstrating GBM linear immunofluorescence staining on kidney biopsy and detecting anti-GBM antibodies in serum. Read More

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http://dx.doi.org/10.5339/qmj.2020.16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7199789PMC

Serological testing in small vessel vasculitis.

Rheumatology (Oxford) 2020 May;59(Supplement_3):iii51-iii54

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

Serological analysis has a central role in the diagnostic work-up of patients with suspected small vessel vasculitis, both for establishing a specific diagnosis and for the monitoring of response to therapy. Autoantibodies can be detected in all forms of primary small vessel vasculitis as well as in the most common forms of secondary vasculitis. For primary vasculitis the most important serological test is for ANCA. Read More

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http://dx.doi.org/10.1093/rheumatology/kez633DOI Listing

Benefit of Howell-Jolly bodies detection: finding of an acquired hyposplenism in a patient with Goodpasture syndrome.

Ann Biol Clin (Paris) 2020 Apr;78(2):191-194

Service d'hématologie biologique, CHU de Rouen, Rouen, France.

Howell-Jolly bodies are intraerythrocytic inclusions corresponding to a small portion of chromatin. Red blood cells that contain these nuclear remnants are removed from the circulation by the spleen. In most cases, presence of Howell-Jolly bodies on a blood smear is the result of functional asplenia and splenectomy. Read More

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http://dx.doi.org/10.1684/abc.2020.1544DOI Listing

Anti-glomerular basement membrane disease: A clinicomorphological study of 16 cases.

Indian J Pathol Microbiol 2020 Apr-Jun;63(2):226-229

Department of Nephrology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India.

Introduction: Antiglomerular basement membrane disease manifests as rapidly progressive glomerulonephritis and alveolar hemorrhage. It encompasses 10-15% of crescentic glomerulonephritis and is associated with poor outcome. In this study, we have elaborated on the clinical details, morphological features, and outcome of anti-GBM glomerulonephritis. Read More

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http://dx.doi.org/10.4103/IJPM.IJPM_712_18DOI Listing

Human leucocyte antigen-associated anti-glomerular basement membrane disease in siblings.

Clin Kidney J 2020 Apr 3;13(2):261-262. Epub 2019 Jun 3.

Department of Renal Medicine, Royal Preston Hospital, Preston, UK.

We report a case of anti-glomerular basement membrane (GBM) disease in association with human leucocyte antigen (HLA) DRB1 15:01. A 71-year-old woman presented with oligoanuric acute kidney injury accompanied by high titre anti-GBM antibodies. Renal biopsy revealed a severe crescentic glomerulonephritis. Read More

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http://dx.doi.org/10.1093/ckj/sfz058DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7147311PMC

Anti-glomerular basement membrane disease complicated with posterior reversible encephalopathy syndrome and subcortical cerebral hemorrhage: a case report and review of the literature.

CEN Case Rep 2020 Apr 10. Epub 2020 Apr 10.

Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 8128582, Japan.

A 71-year-old woman was hospitalized for the treatment of fatigue, fever, and cough. On admission, she showed increased serum inflammation markers, severe anemia, pulmonary hemorrhage, and advanced acute kidney injury requiring hemodialysis. Her serum anti-glomerular basement membrane (GBM) antibody titer was found to be extremely high on the 7th hospital day. Read More

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http://dx.doi.org/10.1007/s13730-020-00473-xDOI Listing

Idiopathic Pulmonary Hemosiderosis as a Mimic of Pulmonary Vasculitis: A Case Report and Review of the Literature.

Curr Allergy Asthma Rep 2020 Apr 4;20(5):13. Epub 2020 Apr 4.

Duke Pediatric Rheumatology, Box 3212 2301 Erwin Rd., Durham, NC, 27705, USA.

Purpose Of Review: Idiopathic pulmonary hemosiderosis (IPH) is one of the rarest and least understood causes of pulmonary hemorrhage in children. Illustrated by a complex case presentation, we discuss the clinical manifestations, diagnosis, pathology, proposed etiologies, and treatment of this rare disease. We also compare IPH with anti-glomerular basement membrane antibody syndrome (anti-GBM disease), another rare causes of pediatric pulmonary hemorrhage. Read More

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http://dx.doi.org/10.1007/s11882-020-00907-7DOI Listing

Use of Rituximab in Management of Rapidly Progressive Glomerulonephritis.

Cureus 2020 Jan 30;12(1):e6820. Epub 2020 Jan 30.

Internal Medicine, California Institute of Behavioral Neurosciences and Psychology, Fairfield, USA.

Rapidly progressive glomerulonephritis (RPGN) is a form of glomerulonephritis characterized by loss of renal function within weeks. Although a variety of underlying causes can trigger RPGN, the ultimate pathologic mechanism is the podocyte and epithelial activation leading to the crescent formation. Rituximab has been increasingly and successfully used for autoimmune conditions in recent years. Read More

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http://dx.doi.org/10.7759/cureus.6820DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7051118PMC
January 2020

[Successful rituximab treatment of recurrent glomerulonephritis associated with antibodies against the glomerular basement membrane].

Internist (Berl) 2020 Apr;61(4):416-423

Abteilung Nephrologie, Universitätsklinikum Regensburg, Franz-Josef-Strauß-Allee 11, 93053, Regensburg, Deutschland.

This article presents a case of recurrent anti-GBM disease (with antibodies against the glomerular basement membrane [GBM]) in a 17-year-old patient successfully treated with rituximab. Kidney biopsy with detection of linear deposition of immunoglobulin G (IgG) along the basement membrane is the diagnostic gold standard, which should be accompanied by serological testing. However, standard assays for the detection of anti-GBM antibodies have a high rate of false-negative results. Read More

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http://dx.doi.org/10.1007/s00108-020-00773-5DOI Listing

Severe Infection in Anti-Glomerular Basement Membrane Disease: A Retrospective Multicenter French Study.

J Clin Med 2020 Mar 4;9(3). Epub 2020 Mar 4.

Department of Nephrology, Dialysis, and Transplantation, University of Picardie Jules Verne, Amiens University Hospital, F-80054 Amiens, France.

In patients presenting with anti-glomerular basement membrane (GBM) disease with advanced isolated kidney involvement, the benefit of intensive therapy remains controversial due to adverse events, particularly infection. We aim to describe the burden of severe infections (SI) (requiring hospitalization or intravenous antibiotics) and identify predictive factors of SI in a large cohort of patients with anti-GBM disease. Among the 201 patients (median [IQR] age, 53 [30-71] years) included, 74 had pulmonary involvement and 127 isolated glomerulonephritis. Read More

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http://dx.doi.org/10.3390/jcm9030698DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7141378PMC

Curcumin Attenuates Both Acute and Chronic Immune Nephritis.

Int J Mol Sci 2020 Mar 4;21(5). Epub 2020 Mar 4.

Department of Biomedical Engineering, University of Houston, Houston, TX 77204, USA.

Curcumin is known to have immunomodulatory potential in addition to anti-oxidant, anti-inflammatory and anti-carcinogenic effects. The aim of the present study is to investigate the therapeutic effects of curcumin on immune-mediated renal disease in an anti-glomerular basement membrane (GBM) model (representing acute kidney Injury, AKI) and murine lupus model (representing chronic kidney disease, CKD). In the AKI model, female anti-GBM 129/svj mice were administered with curcumin right before disease induction. Read More

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http://dx.doi.org/10.3390/ijms21051745DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7084772PMC

Impact of ANCA-Associated Vasculitis on Outcomes of Hospitalizations for Goodpasture's Syndrome in the United States: Nationwide Inpatient Sample 2003-2014.

Medicina (Kaunas) 2020 Mar 1;56(3). Epub 2020 Mar 1.

Division of Nephrology, Department of Medicine, University of Mississippi Medical Center, Jackson, MS 39216, USA.

: Goodpasture's syndrome (GS) is a rare, life-threatening autoimmune disease. Although the coexistence of anti-neutrophil cytoplasmic antibody (ANCA) with Goodpasture's syndrome has been recognized, the impacts of ANCA vasculitis on mortality and resource utilization among patients with GS are unclear. : We used the National Inpatient Sample to identify hospitalized patients with a principal diagnosis of GS from 2003 to 2014 in the database. Read More

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http://dx.doi.org/10.3390/medicina56030103DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7142422PMC
March 2020
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Crescentic glomerulonephritis in children.

Pediatr Nephrol 2020 May 12;35(5):829-842. Epub 2020 Feb 12.

Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany.

Background: To date, there is insufficient knowledge about crescentic glomerulonephritis (cGN), the most frequent immunologic cause of acute kidney injury in children.

Methods: Over a period of 16 years, we retrospectively analyzed kidney biopsy results, the clinical course, and laboratory data in 60 pediatric patients diagnosed with cGN.

Results: The underlying diseases were immune complex GN (n = 45/60, 75%), including IgA nephropathy (n = 19/45, 42%), lupus nephritis (n = 10/45, 22%), Henoch-Schoenlein purpura nephritis (n = 7/45, 16%) and post-infectious GN (n = 7/45, 16%), ANCA-associated pauci-immune GN (n = 10/60, 17%), and anti-glomerular basement-membrane GN (n = 1/60, 2%). Read More

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http://dx.doi.org/10.1007/s00467-019-04436-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7096391PMC

Goodpasture syndrome and hemorrhage after renal biopsy: A case report.

World J Clin Cases 2020 Jan;8(2):404-409

Department of Nephrology, Shenzhen Longhua District Central Hospital, Shenzhen 518110, Guangdong Province, China.

Background: Goodpasture syndrome (GS) is a rare disease, the morbidity of which is estimated to be 0.5-0.8 per million per year. Read More

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http://dx.doi.org/10.12998/wjcc.v8.i2.404DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7000945PMC
January 2020

Inpatient Burden and Mortality of Goodpasture's Syndrome in the United States: Nationwide Inpatient Sample 2003-2014.

J Clin Med 2020 Feb 6;9(2). Epub 2020 Feb 6.

Division of Nephrology, Department of Medicine, University of Mississippi Medical Center, Jackson, MS 39216, USA.

Goodpasture's syndrome is a rare, life-threatening, small vessel vasculitis. Given its rarity, data on its inpatient burden and resource utilization are lacking. We conducted this study aiming to assess inpatient prevalence, mortality, and resource utilization of Goodpasture's syndrome in the United States. Read More

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http://dx.doi.org/10.3390/jcm9020455DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7074028PMC
February 2020

What triggered massive hemoptysis in Goodpasture syndrome with isolated pulmonary involvement in a 14-year-old boy?

Turk J Pediatr 2019 ;61(4):611-614

Department of Pediatric Intensive Care Unit, Gazi University Faculty of Medicine, Ankara, Turkey.

Şişmanlar-Eyüboğlu T, Aslan AT, Gezgin-Yıldırım D, Buyan N, Emeksiz S, Kalkan G. What triggered massive hemoptysis in Goodpasture syndrome with isolated pulmonary involvement in a 14-year-old boy? Turk J Pediatr 2019; 61: 611-614. Goodpasture syndrome is a rare, autoimmune disease associated with the development of antibodies against a specific antigen both in glomerular basement membrane and alveolar basement membrane. Read More

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http://dx.doi.org/10.24953/turkjped.2019.04.022DOI Listing

Rituximab use in adult glomerulopathies and its rationale.

J Bras Nefrol 2020 Mar 20;42(1):77-93. Epub 2019 Dec 20.

Departamento de Nefrologia, Hospital Espírito Santo de Évora, Évora, Portugal.

Glomerulopathies are one of the leading causes of end-stage renal disease. In the last years, clinical research has made significant contributions to the understanding of such conditions. Recently, rituximab (RTX) has appeared as a reasonably safe treatment. Read More

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http://dx.doi.org/10.1590/2175-8239-JBN-2018-0254DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7213927PMC

Membranous Glomerulonephritis With Crescents.

Kidney Int Rep 2019 Nov 13;4(11):1577-1584. Epub 2019 Aug 13.

Centre for Inflammatory Disease, Division of Immunology and Inflammation, Department of Medicine, Imperial College London, London, UK.

Introduction: Membranous glomerulonephritis (MGN) is rarely associated with necrotizing and crescentic glomerulonephritis (NCGN).

Methods: We report the clinical and pathologic findings in 15 patients with MGN and NCGN associated with anti-neutrophil cytoplasm antibodies (ANCAs), anti-glomerular basement membrane (GBM), or anti-phospholipase A2 receptor (PLA2R) antibodies.

Results: The cohort consisted of 15 patients: 7 males and 8 females with a median age of 63 years (range: 18-79). Read More

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http://dx.doi.org/10.1016/j.ekir.2019.07.021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6933443PMC
November 2019

Comparison of the performance of a chemiluminescence assay and an ELISA for detection of anti-GBM antibodies.

Ren Fail 2020 Nov;42(1):48-53

Renal Division, Department of Medicine, Peking University First Hospital, Beijing, PR. China.

Autoantibodies to the α3 chain noncollagen 1 domain of type IV collagen (α3(IV)NC1) are a serological hallmark in the diagnosis of anti-glomerular basement membrane (GBM) disease. The objective of our study was to compare the performance of anti-glomerular basement membrane (GBM) antibody detection by chemiluminescence immunoassay (CIA) and by enzyme-linked immunosorbent assays (ELISAs). Sera from outpatients who were suspected to have anti-GBM disease and 31 patients with biopsy-proven anti-GBM disease were collected. Read More

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http://dx.doi.org/10.1080/0886022X.2019.1702056DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6968565PMC
November 2020

[ANCA and anti-MBG double-positive vasculitis: An update on the clinical and therapeutic specificities and comparison with the two eponymous vasculitis].

Rev Med Interne 2020 Jan 12;41(1):21-26. Epub 2019 Dec 12.

Service de médecine interne et immunologie clinique, UNICAEN, Normandie Université, CHU de Caen Normandie, 14000 Caen, France.

Double-positive vasculitis with anti-polynuclear cytoplasm (ANCA) and anti-glomerular basement membrane (GBM) antibodies is a rare entity of systemic vasculitis defined by the presence of ANCA and anti-GBM antibodies. The gradual accumulation of clinical and therapeutic data shows the usefulness of identifying and differentiating this entity from the two vasculitis respectively associated with the isolated presence of each of these two antibodies. Indeed, the double-positive ANCA and anti-GBM vasculitis appears to associate the characteristics of the demography and the extra-renal and pulmonary involvement of the ANCA-associated vasculitis on the one hand, and of the histological type and severe renal prognosis of the anti-MBG vasculitis on the other hand, with the renal involvement which is the only involvement consistently observed in double-positive vasculitis. Read More

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http://dx.doi.org/10.1016/j.revmed.2019.10.334DOI Listing
January 2020

Atypical anti-glomerular basement membrane disease presenting as macroscopic haematuria, loin pain and acute kidney injury after intensive exercise.

Clin Kidney J 2019 Dec 23;12(6):801-802. Epub 2019 Apr 23.

Department of Nephrology - Transplantation, CHU de la Réunion Felix Guyon, Saint Denis, Réunion, France.

We report a 35-year-old man who suffered from recurrent macroscopic haematuria after intensive exercise. One episode was associated with bilateral loin (flank) pain and severe acute kidney injury. His kidney biopsy revealed an atypical anti-glomerular basement membrane (GBM) disease typified by bright linear GBM staining for monotypic immunoglobulin G but without a diffuse crescentic phenotype and no circulating anti-GBM antibody. Read More

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http://dx.doi.org/10.1093/ckj/sfz044DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6885666PMC
December 2019

Plasma exchanges. A still effective treatment.

Authors:
Loïc Guillevin

Presse Med 2019 11;48(11 Pt 2):317-318

Université Paris Descartes, Hôpital Cochin, Department of Internal Medicine, Referral Center for Rare Systemic and Autoimmune Diseases: Vasculitis and Scleroderma, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.lpm.2019.11.007DOI Listing
November 2019

Double positivity for antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane antibody could predict end-stage renal disease in ANCA-associated vasculitis: a monocentric pilot study.

Clin Rheumatol 2020 Mar 4;39(3):831-840. Epub 2019 Dec 4.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.

Objectives: To investigate the detection rate of double positivity for antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) antibody at diagnosis and its clinical implication during follow-up in Korean patients with ANCA-associated vasculitis (AAV).

Methods: We retrospectively reviewed the medical records of 96 Korean AAV patients. We obtained data at diagnosis and assessed the poor outcomes of AAV such as all-cause mortality, relapse and end-stage renal disease (ESRD). Read More

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http://dx.doi.org/10.1007/s10067-019-04854-1DOI Listing

A rare case of Goodpasture syndrome concomitant with bleeding jejunal Dieulafoy's lesion.

Clin J Gastroenterol 2020 Jun 30;13(3):382-385. Epub 2019 Nov 30.

Department of Nephrology, Kyoto Okamoto Memorial Hospital, Kyoto, Japan.

An 81-year-old man was diagnosed with Goodpasture syndrome (GS) because he met the criteria of positive anti-GBM antibodies, rapid progressive glomerulonephritis and pulmonary hemorrhage. After starting plasmapheresis and steroid pulse therapy, he experienced tarry stool and contrast-enhanced CT revealed an aneurysmal finding in the jejunum. Paroral enteroscopy showed a jejunal Dieulafoy's lesion (DL) with gush-out hemorrhage. Read More

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http://dx.doi.org/10.1007/s12328-019-01078-3DOI Listing

[Review on anti-glomerular basement membrane disease or Goodpasture's syndrome].

Rev Med Interne 2020 Jan 24;41(1):14-20. Epub 2019 Nov 24.

Sorbonne Université, UPMC Université Paris 06, UMR 7211, and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), 75005, Paris, France; Inserm, UMR_S 959, 75013, Paris, France; CNRS, FRE3632, 75005, Paris, France; Department of Internal Medicine and Clinical Immunology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, 75013, Paris, France; Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires et de l'Amylose, 94270 Le Kremlin Bicêtre, France.

Anti-glomerular basement membrane (anti-GBM) disease or Goodpasture's syndrome is a small vessel vasculitis affecting the capillary beds of kidneys and lungs. It is an autoimmune disease mediated by autoantibodies targeting the glomerular and alveolar basement membranes, leading to pneumorenal syndrome. It is a rare, monophasic and severe disease, associating rapidly progressive glomerulonephritis and alveolar hemorrhage. Read More

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http://dx.doi.org/10.1016/j.revmed.2019.10.338DOI Listing
January 2020

A new mouse model of anti-GBM disease sheds light on maternal transfer of alloantibodies in glomerular disease.

Kidney Int 2019 Dec;96(6):1272-1274

Division of Nephrology, Department of Pediatric Subspecialties, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy. Electronic address:

Membranous nephropathy and anti-glomerular basement membrane glomerulonephritis are characterized by the deposition of antibodies that recognize specific glomerular epitopes. These antibodies may develop in autoimmune diseases, after exposure to new antigens or after passive maternal transfer, such as in neonatal membranous nephropathy secondary to in utero transfer of anti-neutral endopeptidase protein Igs. In this issue of Kidney International, Abrahamson et al. Read More

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http://dx.doi.org/10.1016/j.kint.2019.08.007DOI Listing
December 2019

Complement alternative pathway in ANCA-associated vasculitis: Two decades from bench to bedside.

Autoimmun Rev 2020 Jan 15;19(1):102424. Epub 2019 Nov 15.

Service de Néphrologie-Dialyse-Transplantation, Université d'Angers, CHU Angers, Angers, France; CRCINA, INSERM, Université de Nantes, Université d'Angers, Angers, France. Electronic address:

Anti-neutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitides (AAVs) are small vessel vasculitides involving predominantly ear-nose-throat, kidneys, lungs and nerves. AAVs are life-threatening diseases, especially in their most severe forms such as necrotizing crescentic glomerulonephritis (GN) and/or intra-alveolar hemorrhage. Unlike immune complex GN or anti-glomerular basement membrane GN, AAVs are classified as pauci-immune GN. Read More

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http://dx.doi.org/10.1016/j.autrev.2019.102424DOI Listing
January 2020

Severe acute respiratory failure due to Sai-rei-to-induced lung injury successfully treated by multi-modal therapy including immunosuppressive therapy, plasma exchange, and intravenous immunoglobulin: A case report.

Respir Med Case Rep 2019 16;28:100955. Epub 2019 Oct 16.

Department of Pulmonology, Kameda Medical Center, 929 Higashi-cho, Kamogawa-shi, Chiba, 296-8602, Japan.

Corticosteroid therapy may not be enough to control pneumonitis in some cases of severe drug-induced lung injury (DLI); however, an advanced treatment strategy for such cases is lacking. Here, we report the case of an 88-year-old man who presented with severe DLI, caused by Sai-rei-to. The patient visited our hospital complaining of progressive dyspnea. Read More

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http://dx.doi.org/10.1016/j.rmcr.2019.100955DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6831833PMC
October 2019

Plasma exchange in anti-glomerular basement membrane disease.

Presse Med 2019 Nov 5;48(11 Pt 2):328-337. Epub 2019 Nov 5.

Imperial College London, Hammersmith Campus, Department of Medicine, Du Cane Road, W12 0NN London, UK.

Anti-glomerular basement membrane (GBM) disease is a rare autoimmune vasculitis characterised by antibodies directed against the non collagenous (NC1) domain of the α3 chain of type 4 collagen (α3(IV)NC1). Clinical features are typically of a rapidly progressive glomerulonephritis (RPGN) with or without pulmonary haemorrhage. Treatment aims to rapidly remove circulating autoantibodies with plasma exchange and prevent further antibody production and suppress inflammation using immunosuppression and corticosteroids. Read More

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http://dx.doi.org/10.1016/j.lpm.2019.03.017DOI Listing
November 2019

Double Trouble.

N Engl J Med 2019 Nov;381(19):1854-1860

From the Veterans Affairs (VA) Ann Arbor Medical Center (C.S., S.S., P.G.) and the University of Michigan Medical School (C.S., S.S., E.A.F., P.G.) - both in Ann Arbor; and the University of California, San Francisco, School of Medicine and the San Francisco VA Medical Center - both in San Francisco (G.D.).

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http://dx.doi.org/10.1056/NEJMcps1810654DOI Listing
November 2019
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Rituximab in treatment of anti-GBM antibody glomerulonephritis: A case report and literature review.

Medicine (Baltimore) 2019 Nov;98(44):e17801

Department of Nephrology & Hypertension, Dokkyo Medical University, Mibu, Tochigi.

Rationale: Anti-glomerular basement membrane (GBM) disease is a T cell-mediated disease that has a poor prognosis with conventional therapy. We tested rituximab as a primary therapy to reduce anti-GBM antibody produced by B cells.

Patient Concerns: A 53-year old woman with complaints of a fever, headache and abdominal discomfort showed renal failure with elevated anti-GBM antibody, and renal biopsy revealed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) 1 deposition along GBM. Read More

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http://dx.doi.org/10.1097/MD.0000000000017801DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6946414PMC
November 2019

IdeS in anti-glomerular basement membrane disease: Is this the new deal?

Kidney Int 2019 Nov;96(5):1068-1070

Urgences Néphrologiques et Transplantation Rénale, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris, France; Sorbonne Université, Inserm, UMR_S1155, F-75020, Paris, France.

IdeS, a proteinase from Streptococcus pyogenes, cleaves IgG antibodies with a unique specificity. Herein, the authors report the dramatic efficacy of IdeS on the levels of anti-glomerular membrane autoantibodies in 3 three patients with Goodpasture disease refractory to standard therapy. The levels of anti-glomerular membrane autoantibodies were reduced to near-zero levels within 2 hours of the injection. Read More

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http://dx.doi.org/10.1016/j.kint.2019.08.017DOI Listing
November 2019

A case of anti-neutrophil cytoplasmic antibody-associated vasculitis with anti-glomerular basement membrane antibodies that was successfully treated with mizoribine as a safe and effective remission maintenance therapy with prednisolone and plasma exchange.

CEN Case Rep 2020 Feb 14;9(1):42-47. Epub 2019 Oct 14.

Department of Internal Medicine, Faculty of Medicine, Saga University, Saga, Japan.

We herein report the case of myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with anti-glomerular basement membrane (anti-GBM) antibody positivity that successfully treated with mizoribine (MZR) as an immunosuppressive drug for remission maintenance therapy after the initiation of dialysis in addition to plasma exchange (PE) and glucocorticoid treatment to control the disease condition. A 79-year-old woman developed serious renal dysfunction and pulmonary alveolar hemorrhaging due to MPO-ANCA and anti-GBM antibody double-positive vasculitis. She was started on hemodialysis and was treated with methylprednisolone (m-PSL) pulse therapy with PE, followed by oral prednisolone (PSL). Read More

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http://dx.doi.org/10.1007/s13730-019-00423-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6990334PMC
February 2020
1 Read

Management of an Adult with Goodpasture's Syndrome Following Brain Trauma with Extracorporeal Membrane Oxygenation: A Case Report.

Chin Med Sci J 2019 Sep;34(3):226-229

Institute of Clinical Nutrition, Guangzhou Red Cross Hospital, Medical College, Jinan University, Guangzhou 510220, China.

A 22-year-old man suffered from acute pulmonary hemorrhage and deteriorated renal function occurred within 3 days after traumatic brain injury. Mechanical ventilation cannot correct his severe hypoxemia, therefore, venoarterial extracorporeal membrane oxygenation (VA-ECMO) support was initiated and finally resolved his hypoxemia. Concomitantly, continuous renal replacement therapy was performed to improve his kidney function. Read More

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http://dx.doi.org/10.24920/003478DOI Listing
September 2019
2 Reads

The IgG-degrading enzyme of Streptococcus pyogenes causes rapid clearance of anti-glomerular basement membrane antibodies in patients with refractory anti-glomerular basement membrane disease.

Kidney Int 2019 Nov 1;96(5):1234-1238. Epub 2019 Aug 1.

Departments of Nephrology and Medical and Health Sciences, Linköping University, Linköping, Sweden.

In anti-glomerular basement membrane (anti-GBM) disease, IgG class autoantibodies induce rapidly progressive glomerulonephritis. Regrettably, many patients are diagnosed at a late stage when even intensive conventional treatment fails to restore renal function The endopeptidase IdeS (Immunoglobulin G degrading enzyme of Streptococcus pyogenes) (imliflidase) rapidly cleaves all human IgG subclasses into F(ab') and Fc fragments. We received permission to treat three patients with refractory anti-GBM nephritis without pulmonary involvement on a compassionate basis. Read More

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http://dx.doi.org/10.1016/j.kint.2019.06.019DOI Listing
November 2019
2 Reads

The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis.

Authors:
Elena Csernok

Antibodies (Basel) 2019 May 1;8(2). Epub 2019 May 1.

Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Center Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, 73230 Kirchheim-Teck, Germany.

Considerable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the diagnosis, prognosis, and monitoring of small-vessel vasculitis. ANCA-directed proteinase 3 (PR3-) or myeloperoxidase (MPO-) are sensitive and specific serologic markers for ANCA-associated vasculitides (AAV), anti-GBM antibodies are highly specific for the patients with anti-GBM antibody disease (formerly Goodpasture's syndrome), and autoantibodies to C1q are characteristic of hypocomlementemic urticarial vasculitis syndrome (HUVS; anti-C1q vasculitis). The results of a current EUVAS study have led to changes in the established strategy for the ANCA testing in small-vessel vasculitis. Read More

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http://dx.doi.org/10.3390/antib8020031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6640716PMC
May 2019
2 Reads

A case of bizarre posttransplant anti-glomerular basement membrane disease.

Cent Eur J Immunol 2019 30;44(2):210-213. Epub 2019 Jul 30.

Department of Immunology, Poznan University of Medical Sciences, Poland.

The non-collagenous (NC1) domain of α3 and α5 chains of type IV collagen are eminent targets of abnormal immune response in anti-glomerular basement membrane (anti-GBM) disease, which can be diagnosed by the presence of strong linear IgG staining along GBM detected by direct immunofluorescence. The presence of linear GBM fixation in renal allograft is a rare finding. We observed a 33-year-old male with de novo renal failure in a kidney transplant. Read More

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http://dx.doi.org/10.5114/ceji.2019.87074DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6745540PMC
July 2019
8 Reads
0.358 Impact Factor

Maternal alloimmune IgG causes anti-glomerular basement membrane disease in perinatal transgenic mice that express human laminin α5.

Kidney Int 2019 Dec 10;96(6):1320-1331. Epub 2019 Jul 10.

Department of Microbiology, Immunology and Physiology, Meharry Medical College, Nashville, Tennessee, USA.

Mammalian immune systems are not mature until well after birth. However, transfer of maternal IgG to the fetus and newborn usually provides immunoprotection from infectious diseases. IgG transfer occurs before birth in humans across the placenta and continues after birth across the intestine in many mammalian species, including rodents. Read More

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http://dx.doi.org/10.1016/j.kint.2019.06.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6875631PMC
December 2019
4 Reads

Concurrent Anti-glomerular Basement Membrane Nephritis and IgA Nephropathy.

J Pathol Transl Med 2019 Nov 16;53(6):399-402. Epub 2019 Sep 16.

Department of Pathology, Chungnam National University School of Medicine, Daejeon, Republic of Korea.

Anti-glomerular basement membrane (GBM) nephritis is characterized by circulating anti-GBM antibodies and crescentic glomerulonephritis (GN) with deposition of IgG along the GBM. In a limited number of cases, glomerular immune complexes have been identified in anti-GBM nephritis. A 38-year-old female presented azotemia, hematuria, and proteinuria without any pulmonary symptoms. Read More

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http://dx.doi.org/10.4132/jptm.2019.08.05DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6877440PMC
November 2019
1 Read

[What are the indications for rescue procedures? : Systemic rheumatic diseases in the intensive care unit].

Z Rheumatol 2019 Dec;78(10):955-966

Vaskulitiszentrum Süd, Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken - Akademisches Lehrkrankenhaus, Universität Tübingen, Eugenstr. 3, 73230, Kirchheim u. Teck, Deutschland.

Severe, organ-threatening and life-threatening manifestations of inflammatory rheumatic diseases, such as diffuse alveolar hemorrhage in the context of small vessel vasculitis, sometimes inadequately respond to immunosuppressive treatment. In the case of an immanent or already occurring organ failure, immunosuppressive treatment may need to be supplemented with rapidly effective rescue treatment procedures. Due to the rarity of many rheumatic diseases, the evidence for the use of rescue treatment, such as plasmapheresis, extracorporeal membrane oxygenation (ECMO) and the administration of intravenous immunoglobulins (IVIG), is relatively low for many indications. Read More

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http://dx.doi.org/10.1007/s00393-019-00687-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7101899PMC
December 2019
2 Reads

A "double-seropositive" Vasculitis for antiglomerular basement membrane antibodies and antineutrophil cytoplasmic antibody, and multiple myeloma, all three with simultaneous diagnosis of renal involvement, common pathophysiology, or pure coincidence? first case in the World.

Saudi J Kidney Dis Transpl 2019 Jul-Aug;30(4):953-959

Department of Nephrology-Hemodialysis and Kidney Ttransplantation, University Hospital Mohammed VI, Marrakech, Morocco.

The combination of Goodpasture's disease and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is not exceptional. However, to the best of our knowledge, their association with multiple myeloma (MM) with kidney involvemen thas not been described. We report probably the first case of double-seropositive vasculitis for antiglomerular basement membrane (GBM) antibodies and ANCA associated with MM with renal involvement. Read More

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http://dx.doi.org/10.4103/1319-2442.265473DOI Listing
February 2020
1 Read

Prognostic Factors in Anti-glomerular Basement Membrane Disease: A Multicenter Study of 119 Patients.

Front Immunol 2019 18;10:1665. Epub 2019 Jul 18.

Inflammation-Immunopathology-Biotherapy Department (DHU i2B), Sorbonne Université, UPMC Univ Paris 06, UMR 7211, Paris, France.

We report the overall and renal outcome in a French nationwide multicenter cohort of 119 patients with anti-glomerular basement membrane (anti-GBM) disease. Sixty-four patients (54%) had an exclusive renal involvement, 7 (6%) an isolated alveolar hemorrhage and 48 (40%) a combined renal and pulmonary involvement. Initial renal replacement therapy (RRT) was required in 78% of patients; 82% received plasmapheresis, 82% cyclophosphamide, and 9% rituximab. Read More

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http://dx.doi.org/10.3389/fimmu.2019.01665DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6662558PMC
July 2019
9 Reads

A Man in His 50s With Hemoptysis, Dyspnea, and Bilateral Patchy Ground-Glass Opacities.

Chest 2019 08;156(2):e41-e45

Division of Pulmonary Sciences and Critical Care Medicine, Rocky Mountain Regional VA Medical Center, Aurora, CO.

Case Presentation: A man in his 50s presented to the ED with a 3-day history of small-volume hemoptysis and new-onset dyspnea. The patient did not have fevers, chills, chest pain, abdominal pain, or changes in urination. His medical history included hypertension, a 35-pack-year active smoking history, and occupational hydrocarbon exposure as a mechanic in the foresting industry. Read More

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http://dx.doi.org/10.1016/j.chest.2019.03.021DOI Listing

Recurrent anti-GBM disease with T-cell large granular lymphocytic leukemia: A case report.

Medicine (Baltimore) 2019 Aug;98(31):e16649

Division of Nephrology.

Rationale: Anti-glomerular basement membrane disease (anti-GBM disease) is a rare small vessel vasculitis caused by autoantibodies directed against the glomerular and alveolar basement membranes. Anti-GBM disease is usually a monophasic illness and relapse is rare after effective treatment. This article reports a case of coexistence of recurrent anti-GBM disease and T-cell large granular lymphocytic (T-LGL) leukemia. Read More

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http://dx.doi.org/10.1097/MD.0000000000016649DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6708844PMC
August 2019
6 Reads

Rare case of atypical crescentic glomerulonephritis and interstitial lung disease with negative anti-GBM antibody and positive anti-MPO antibody.

BMJ Case Rep 2019 Aug 1;12(8). Epub 2019 Aug 1.

Department of Medicine, Northwestern Memorial Hospital, Chicago, Illinois, USA.

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. Read More

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http://dx.doi.org/10.1136/bcr-2019-229256DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6678005PMC
August 2019
8 Reads

[Recurrence of goodpasture syndrome with negative antiglomerular basement antibodies. Report of one case].

Rev Med Chil 2019 Mar;147(3):390-394

Departamento de Nefrología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

Goodpasture Syndrome is described as a single episode disease entity. It is diagnosed with the demonstration of antiglomerular basement (anti-GBM) antibodies in plasma or renal tissue. Although the recurrence of anti-GBM disease is rare, it has been reported in up to 3% of cases. Read More

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http://dx.doi.org/10.4067/S0034-98872019000300390DOI Listing
March 2019
3 Reads
0.373 Impact Factor