T cells in ANCA-associated vasculitis: what can we learn from lesional versus circulating T cells?
Anti-neutrophil cytoplasmic antibody (ANCA) - associated vasculitis (AAV) is a life-threatening autoimmune disease characterized by an antibody-mediated glomerulonephritis and necrotizing vasculitis. Apart from antibodies, T cells are also involved in disease pathogenesis. This review stresses the hallmarks of T cell-mediated pathology in AAV and highlights the characteristics of lesional and circulating T cells in the immune response in AAV. Circulating effector T-cell populations are expanded and are in a persistent state of activation. Circulating regulatory T-cell subsets are less well characterized but seem to be impaired in function. Lesional effector T cells are present in granulomas, vasculitic lesions, and nephritis. Lesional T cells usually show pro-inflammatory properties and promote granuloma formation. Apart from T cells, dendritic cells are abundantly present at the sites of inflammation and locally orchestrate the immune response. Targeting the above-mentioned T cell-mediated disease mechanisms will potentially provide powerful therapeutic tools for AAV.
Wilde
Benjamin
B
Department of Internal Medicine, Division of Clinical and Experimental Immunology, University Hospital Maastricht, Universiteitssingel 50, PO Box 616, 6200 MD Maastricht The Netherlands. benjamin.wilde@immuno.unimaas.nl.
Thewissen
Marielle
M
Damoiseaux
Jan
J
van Paassen
Pieter
P
Witzke
Oliver
O
Tervaert
Jan Willem Cohen
JW
D000818:Animals; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000097:blood* / Q000276:immunology* / Q000473:pathology; D006801:Humans; D013601:T-Lymphocytes / Q000276:immunology* / Q000473:pathology