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Title: Myeloperoxidase-specific antineutrophil cytoplasmic antibody-associated vasculitis.
Authors: Arnold S.;Kitching A.R. ;Witko-Sarsat V.;Wiech T.;Specks U.;Klapa S.;Comduhr S.;Stahle A.;Muller A.;Lamprecht P.
Monash Health Department(s): Centre for Inflammatory Diseases at Monash Health
Paediatric - Nephrology
Institution: (Arnold, Klapa, Comduhr, Stahle, Muller, Lamprecht) Department of Rheumatology and Clinical Immunology, University of Lubeck, Lubeck, Germany
(Kitching) Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Clayton, VIC, Australia
(Kitching) Departments of Nephrology and Pediatric Nephrology, Monash Health, Clayton, VIC, Australia
(Witko-Sarsat) INSERM U1016, Institut Cochin, CNRS UMR8104, Universite Paris Cite et Laboratoire d'Excellence INFLAMEX, Paris, France
(Wiech) Section of Nephropathology, Institute of Pathology, University Hospital Hamburg-Eppendorf, Hamburg, Germany
(Specks) Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, United States
Issue Date: 24-Apr-2024
Copyright year: 2024
Publisher: Elsevier Ltd
Place of publication: United Kingdom
Publication information: The Lancet Rheumatology. 6(5) (pp e300-e313), 2024. Date of Publication: May 2024.
Journal: The Lancet Rheumatology
Abstract: Myeloperoxidase (MPO)-specific antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (MPO-ANCA-associated vasculitis) is one of two major ANCA-associated vasculitis variants characterised by systemic necrotising vasculitis with few or no immune deposits. MPO-ANCA-associated vasculitis predominantly affects small blood vessels and, in contrast to its counterpart proteinase 3-ANCA-associated vasculitis, is generally not associated with granulomatous inflammation. The kidneys and lungs are the most commonly affected organs. The pathogenesis of MPO-ANCA-associated vasculitis is characterised by loss of tolerance to the neutrophil enzyme MPO. This loss of tolerance leads to a chronic immunopathological response where neutrophils become both the target and effector of autoimmunity. MPO-ANCA drives neutrophil activation, leading in turn to tissue and organ damage. Clinical trials have improved the therapeutic approach to MPO-ANCA-associated vasculitis. However, there remains substantial unmet need regarding relapse frequency, toxicity of current treatment, and long-term morbidity. In this Series paper, we present the current state of research regarding pathogenesis, diagnosis, and treatment of MPO-ANCA-associated vasculitis.Copyright © 2024 Elsevier Ltd
Type: Review
Subjects: ANCA associated vasculitis
Type of Clinical Study or Trial: Review article (e.g. literature review, narrative review)
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