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.
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.
Benjamin
B
Wilde
Marielle
M
Thewissen
Jan
J
Damoiseaux
Pieter
P
van Paassen
Oliver
O
Witzke
Jan Willem Cohen
JW
Tervaert
D000818:Animals; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000097:blood* / Q000276:immunology* / Q000473:pathology; D006801:Humans; D013601:T-Lymphocytes / Q000276:immunology* / Q000473:pathology