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http://dx.doi.org/10.1136/annrheumdis-2020-217416DOI Listing

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Background: Cardiovascular disease (CVD) is a leading cause of death in ANCA-associated vasculitis (AAV). Screening and primary cardiovascular prevention may improve outcomes.

Methods: We identified patients in the 2002-2019 Mass General Brigham AAV cohort with thoracic CT scans obtained for other clinical purposes.

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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are autoimmune inflammatory small-vessel disorders with potentially life-threatening organ manifestations. Recent disease definitions and classification criteria allow distinction between granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and non-granulomatous microscopic polyangiitis (MPA). The discovery of ANCA-autoantibodies directed against proteolytic enzymes of neutrophil granules-has enabled earlier diagnosis of AAV and paved the way to stage-adapted treatments.

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Article Synopsis
  • The study evaluated various ANCA detection methods in a group of 147 patients with untreated ANCA-associated vasculitis, focusing primarily on myeloperoxidase (MPO)-ANCA.
  • Different testing methods were compared, including immunofluorescence (IIF) and several antigen-specific immunoassays (ELISA, CLEIA, FEIA, LTIA), revealing varying sensitivities and specificities for diagnosing AAV.
  • Results indicated that while antigen-specific immunoassays performed well overall, using dual IIF and these immunoassays had limited benefits, and standardizing ANCA measurements across different methods remained challenging.
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Introduction: Relapses in ANCA-associated vasculitis (AAV) increase the incidence of end-organ damage and their prevention requires prolonged immunosuppressive therapy. Rituximab, a type I anti-CD20 B cell depleting monoclonal antibody, is the current standard of care for induction of disease remission. Rituximab is not always effective and is associated with a high subsequent relapse risk.

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Introduction: A significant number of patients with antineutrophil cytoplasmic antibodies (ANCA)- associated vasculitis (AAV) with glomerulonephritis (AAV-GN) still progress to end-stage kidney disease (ESKD, estimated glomerular filtration rate [eGFR] <15 ml/min per 1.73 m) despite advances in remission-induction treatment.

Methods: This is a retrospective cohort study on myeloperoxidase (MPO)-ANCA or proteinase 3 (PR3)-ANCA positive patients with AAV (microscopic polyangiitis, MPA; or granulomatosis with polyangiitis, GPA) and eGFR <15 ml/min per 1.

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