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Vasculitis ANCA

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https://www.readbyqxmd.com/read/28076879/igm-antibodies-to-proteinase-3-in-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#1
J M Clain, A M Hummel, J H Stone, F C Fervenza, G S Hoffman, C G M Kallenberg, C A Langford, W J McCune, P A Merkel, P A Monach, P Seo, R F Spiera, E W St Clair, S R Ytterberg, And U Specks
Antineutrophil cytoplasmic antibodies (ANCA) appear to play an important role in the pathogenesis of ANCA-associated vasculitis (AAV). Yet, ANCA alone are not sufficient to generate disease, and some evidence suggests that infectious triggers may serve as inciting events for AAV disease activity. Antibodies of the IgM isotype often serve as markers of recent infection, and IgM ANCA have previously been identified in patients with AAV, though the frequency and clinical relevance of IgM ANCA is not well established...
January 11, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28074992/-antineutrophil-cytoplasmic-antibody-anca-associated-renal-vasculitis-and-pregnancy-report-of-one-case
#2
Jorge Vega, Gonzalo P Méndez
ANCA mediated vasculitis mainly occur between the fourth and fifth decade of life; therefore, it is very uncommon to see pregnant patients with the disease. Vasculitis may affect significantly the course of pregnancy; in turn pregnancy can change the course of vasculitis. We report a 20 years old woman with ANCA-mediated renal vasculitis lasting 10 years who consulted with a pregnancy of 15 weeks. She was in remission and had amenorrhea attributed to ovarian toxicity due to cyclophosphamide. Pregnancy had an uneventful course with spontaneous delivery at the 37th week, giving birth to a healthy newborn...
October 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/28065613/anca-negative-churg-strauss-syndrome-presenting-as-acute-multiple-cerebral-infarcts-a-case-report
#3
Klearchos Psychogios, Ilias Evmorfiadis, Spyros Dragomanovits, Athanasios Stavridis, Konstantinos Takis, Loukas Kaklamanis, Stathis Pantelis
Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA...
January 5, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28062909/paediatric-anti-neutrophil-cytoplasmic-antibody-anca-associated-vasculitis-an-update-on-renal-management
#4
REVIEW
Lucy A Plumb, Louise Oni, Stephen D Marks, Kjell Tullus
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening...
January 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28058500/-paradigm-shift-in-anca-diagnostics-new-international-consensus-recommendations
#5
REVIEW
E Csernok, N Kempiners, B Hellmich
BACKGROUND: Up to now indirect immunofluorescence (IIF) followed by an antigen-specific assay specific for proteinase 3 (PR3) or myeloperoxidase (MPO) has been the standard method for the detection of antineutrophil cytoplasmic antibodies (ANCA). The development of more sensitive and highly specific PR3-ANCA and MPO-ANCA immunoassays for the diagnosis of ANCA-associated vasculitis (AAV) has raised doubts about the two-stage diagnostic strategy currently recommended for ANCA detection...
January 5, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28056474/-granulomatosis-with-polyangiitis
#6
Anja Kerstein, Konstanze Holl-Ulrich, Antje Müller, Gabriela Riemekasten, Peter Lamprecht
Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA)...
January 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28053275/clinical-associations-with-venous-thromboembolism-in-anti-neutrophil-cytoplasm-antibody-associated-vasculitides
#7
Andreas Kronbichler, Johannes Leierer, Gisela Leierer, Gert Mayer, Alina Casian, Peter Höglund, Kerstin Westman, David Jayne
OBJECTIVE: To assess potential associations for the development of venous thromboembolic events in patients with ANCA-associated vasculitides (AAV). METHODS: Four hundred and seventeen patients enrolled to participate in randomized controlled trials conducted by the European Vasculitis Society were identified. Univariate and multivariate analyses were performed to validate previously proposed and identify novel risks associated with venous thromboembolism (VTE) in AAV...
January 3, 2017: Rheumatology
https://www.readbyqxmd.com/read/28050000/the-effects-of-plasma-exchange-on-severe-vasculitis-with-diffuse-alveolar-hemorrhage
#8
Kimihiko Goto, Kentaro Nakai, Hideki Fujii, Shinichi Nishi
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a life-threatening disease characterized by rapidly progressive glomerulonephritis (RPGN) and diffuse alveolar hemorrhage (DAH). Glucocorticoids and immunosuppressants are commonly used to treat this disease but may induce irreversible side effects, particularly in elderly patients. We herein report the case of a 76-year-old woman with RPGN. After methylprednisolone pulse therapy, DAH occurred, and she required ventilatory support. After plasma exchange, her serum creatinine level improved, and she was discharged with home oxygen therapy...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28049651/epigenetic-modifications-in-anca-associated-vasculitis-potential-for-insights-into-disease-pathogenesis-and-prediction-of-outcome
#9
EDITORIAL
Thomas Oates, Alan D Salama
No abstract text is available yet for this article.
January 3, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28040331/therapeutic-plasma-exchange-in-rheumatic-diseases-a-university-hospital-experience
#10
Juan Pablo Córdoba, Carolina Larrarte, Cristina Estrada, Daniel G Fernández-Ávila
INTRODUCTION: Each day, evidence accumulates related to the use of therapeutic plasma exchange (TPE) in patients with rheumatic diseases. San Ignacio University Hospital has recorded all of the TPE sessions performed by the institution's apheresis group. OBJECTIVE: To describe the TPE experience of patients with rheumatologic diseases in a hospital setting (?). METHODS: Descriptive, observational, retrospective analysis. This study included analyses of the TPE sessions that were performed in patients with rheumatic diseases from November 2009 to November 2013...
December 16, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28032843/employment-work-disability-and-quality-of-life-in-patients-with-anca-associated-vasculitides-the-expovas-study
#11
Lucas Benarous, Benjamin Terrier, Hervé Laborde-Casterot, Alice Bérezné, Bertrand Dunogué, Pascal Cohen, Xavier Puéchal, Luc Mouthon, Lynda Bensefa-Colas, Loic Guillevin
OBJECTIVES: Improved therapeutic strategies for ANCA-associated vasculitis (AAV) have transformed acute and life-threatening diseases into chronic ones responsible for marked morbidity that could impact employment, work disability and quality of life (QoL). We aimed to analyse work, handicaps and QoL of AAV patients and identify their determinants. METHODS: Patients with AAV were included in a cross-sectional study assessing employment, work disability and QoL. Specific and non-specific questionnaires, including SF-36, were sent to patients, and clinical-biological data that could affect QoL and their determinants were analysed...
December 20, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28031441/modular-transcriptional-repertoire-analyses-identify-a-blood-neutrophil-signature-as-a-candidate-biomarker-for-lupus-nephritis
#12
Noémie Jourde-Chiche, Elizabeth Whalen, Bertrand Gondouin, Cate Speake, Vivian Gersuk, Bertrand Dussol, Stephane Burtey, Virginia Pascual, Damien Chaussabel, Laurent Chiche
OBJECTIVE: LN is a severe complication of SLE. Non-invasive biomarkers are needed for identifying patients at risk of a renal flare, for differentiating proliferative from non-proliferative forms and for assessing prognoses for LN. METHODS: We assessed the link between blood transcriptional signatures and LN using blood samples from patients with biopsy-proven LN, extra-renal SLE flares or quiescent SLE. Healthy controls, and control patients with glomerular diseases or bacterial sepsis were included...
December 27, 2016: Rheumatology
https://www.readbyqxmd.com/read/28029757/identification-of-functional-and-expression-polymorphisms-associated-with-risk-for-anti-neutrophil-cytoplasmic-autoantibody-associated-vasculitis
#13
Peter A Merkel, Gang Xie, Paul A Monach, Xuemei Ji, Dominic J Ciavatta, Jinyoung Byun, Benjamin D Pinder, Ai Zhao, Jinyi Zhang, Yohannes Tadesse, David Qian, Matthew Weirauch, Rajan Nair, Alex Tsoi, Christian Pagnoux, Simon Carette, Sharon Chung, David Cuthbertson, John C Davis, Paul F Dellaripa, Lindsy Forbess, Ora Gewurz-Singer, Gary S Hoffman, Nader Khalidi, Curry Koening, Carol A Langford, Alfred D Mahr, Carol McAlear, Larry Moreland, E Philip Seo, Ulrich Specks, Robert F Spiera, Antoine Sreih, E William St Clair, John H Stone, Steven R Ytterberg, James T Elder, Jia Qu, Toshiki Ochi, Naoto Hirano, Jeffrey C Edberg, Ronald J Falk, Christopher I Amos, Katherine A Siminovitch
OBJECTIVE: To identify risk alleles relevant to the cause and biology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: We conducted a genome-wide association and subsequent replication study including 1986 cases of AAV [granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)] and 4723 controls. Meta-analysis of these datasets and functional annotation of identified risk loci were performed and candidate disease variants with unknown functional effects investigated for impact on gene expression and/or protein function...
December 28, 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28029751/the-effect-of-continuous-b-cell-depletion-with-rituximab-on-pathogenic-autoantibodies-and-total-igg-levels-in-anca-vasculitis
#14
Frank B Cortazar, William F Pendergraft, Julia Wenger, Charles T Owens, Karen Laliberte, John L Niles
OBJECTIVE: To evaluate the effect of rituximab on pathogenic autoantibodies and total immunoglobulin levels, and to identify serious adverse events, in patients with ANCA vasculitis treated with continuous B cell depletion. METHODS: We conducted a retrospective analysis of 239 patients with ANCA vasculitis treated with rituximab-induced continuous B cell depletion. Two treatment cohorts were analyzed: an induction group (n= 53) and a maintenance group (n=237). The change in ANCA titers and total immunoglobulin levels over time were evaluated using mixed-effects models...
December 28, 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28002315/a-case-report-and-literature-review-of-churg-strauss-syndrome-presenting-with-myocarditis
#15
Lu Qiao, Dengfeng Gao
BACKGROUND: Churg-Strauss syndrome (CSS) is a multisystem disorder characterized by asthma, prominent peripheral blood eosinophilia, and vasculitis signs. CASE SUMMARY: Here we report a case of CSS presenting with acute myocarditis and heart failure and review the literature on CSS with cardiac involvement. A 59-year-old man with general fatigue, numbness of limbs, and a 2-year history of asthma was admitted to the department of orthopedics. Eosinophilia, history of asthma, lung infiltrates, peripheral neurological damage, and myocarditis suggested the diagnosis of CSS...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27980013/histopathological-classification-and-renal-outcome-in-patients-with-antineutrophil-cytoplasmic-antibodies-associated-renal-vasculitis-a-study-of-186-patients-and-metaanalysis
#16
Yong-Xi Chen, Jing Xu, Xiao-Xia Pan, Ping-Yan Shen, Xiao Li, Hong Ren, Xiao-Nong Chen, Li-Yan Ni, Wen Zhang, Nan Chen
OBJECTIVE: Renal vasculitis is one of the most common manifestations of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) and renal histology is a key predictor of the outcome. A new histopathologic classification was proposed and validated, but the results are still debated. METHODS: We performed a retrospective analysis to validate the histopathologic classification and performed a metaanalysis to evaluate its predictive value. There were 186 patients with ANCA-associated renal vasculitis diagnosed at Ruijin Hospital who were enrolled in the retrospective study...
December 15, 2016: Journal of Rheumatology
https://www.readbyqxmd.com/read/27977428/breaking-down-the-complement-system-a-review-and-update-on-novel-therapies
#17
Yuvaram N V Reddy, Andrew M Siedlecki, Jean M Francis
PURPOSE OF REVIEW: The complement system represents one of the more primitive forms of innate immunity. It has increasingly been found to contribute to pathologies in the native and transplanted kidney. We provide a concise review of the physiology of the complement cascade, and discuss current and upcoming complement-based therapies. RECENT FINDINGS: Current agents in clinical use either bind to complement components directly or prevent complement from binding to antibodies affixed to the endothelial surface...
December 12, 2016: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/27973575/anca-associated-glomerulonephritis-risk-factors-for-renal-relapse
#18
Arda Göçeroğlu, Annelies E Berden, Marta Fiocco, Oliver Floßmann, Kerstin W Westman, Franco Ferrario, Gill Gaskin, Charles D Pusey, E Christiaan Hagen, Laure-Hélène Noël, Niels Rasmussen, Rüdiger Waldherr, Michael Walsh, Jan A Bruijn, David R W Jayne, Ingeborg M Bajema
Relapse in ANCA-associated vasculitis (AAV) has been studied previously, but there are few studies on renal relapse in particular. Identifying patients at high risk of renal relapse may aid in optimizing clinical management. We investigated which clinical and histological parameters are risk factors for renal relapse in ANCA-associated glomerulonephritis (AAGN). Patients (n = 174) were newly diagnosed and had mild-moderate or severe renal involvement. Data were derived from two trials of the European Vasculitis Society: MEPEX and CYCAZAREM...
2016: PloS One
https://www.readbyqxmd.com/read/27939215/the-functional-activities-of-complement-factor-h-are-impaired-in-patients-with-anca-positive-vasculitis
#19
Su-Fang Chen, Feng-Mei Wang, Zhi-Ying Li, Feng Yu, Min Chen, Ming-Hui Zhao
Increasing evidences have demonstrated that the activation of the alternative complement pathway is crucial for the pathogenesis of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Our recent study found that circulating levels of complement factor H (FH), a key regulator of the alternative pathway, were associated with disease activity. In the current study, functional activities of FH were assessed to further explore the potential role of FH in the pathogenesis of AAV. We found that the two patients with ANCA-negative pauci-immune necrotizing crescentic glomerulonephritis exhibited relatively normal functional activities of FH...
December 6, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/27919708/early-interstitial-lung-disease-in-microscopic-polyangiitis-case-report-and-literature-review
#20
Marcos García-Nava, Heidegger Mateos-Toledo, Ana Patricia Georgina Guevara-Canseco, Cesar Eduardo Infante-González, Diego Alberto Reyes-Nava, Emilio Estrada-Castro
Microscopic polyangiitis (MPA) is a systemic disease included in the Chapel Hill 2012 Classification as necrotizing vasculitis affecting capillaries, venules and arterioles. It usually expresses antineutrophil cytoplasmic antibodies (ANCA) and has a perinuclear immunofluorescence pattern and correlation with anti-myeloperoxidase (MPO) antibodies. Capillaritis with alveolar hemorrhage is the most common manifestation of lung disease. Interstitial lung disease (ILD) is uncommon, with usual interstitial pneumonia being the predominant pattern...
December 2, 2016: Reumatología Clinica
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