TY - JOUR
T1 - Myeloperoxidase-specific antineutrophil cytoplasmic antibody-associated vasculitis
AU - Arnold, Sabrina
AU - Kitching, A. Richard
AU - Witko-Sarsat, Veronique
AU - Wiech, Thorsten
AU - Specks, Ulrich
AU - Klapa, Sebastian
AU - Comdühr, Sara
AU - Stähle, Anja
AU - Müller, Antje
AU - Lamprecht, Peter
N1 - Publisher Copyright:
© 2024 Elsevier Ltd
PY - 2024/5
Y1 - 2024/5
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85189159719&partnerID=8YFLogxK
U2 - 10.1016/S2665-9913(24)00025-0
DO - 10.1016/S2665-9913(24)00025-0
M3 - Scientific review articles
AN - SCOPUS:85189159719
SN - 2665-9913
VL - 6
SP - e300-e313
JO - The Lancet Rheumatology
JF - The Lancet Rheumatology
IS - 5
ER -