title: Immunotherapy for antineutrophil cytoplasmic antibody-associated vasculitis: challenging the therapeutic status quo?
abstract: Anti-neutrophil cytoplasmic antibodies (ANCA) are IgG antibodies directed against antigenic constituents of neutrophils contained in the azurophilic granules and monocyte lysosomes. Systemic vasculitis with ANCA [ANCA-associated vasculitides (AAV)] is a subgroup of life-threatening inflammatory disorders affecting small- to medium-sized vessels; immunosuppressants and glucocorticoids represent the current therapeutic mainstay. Although these agents have improved patients' survival, 25% present with severe adverse events, and standard therapy does not sustain remission. Therefore, an unmet need for safer and more effective therapies has prompted interest in biological agents. Continuous advances in the knowledge of AAV pathogenesis are paving the way to new biologicals that are now awaiting testing.
authors.affiliation: Department of Internal Medicine, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, Spain. xavbosch@clinic.ub.es <xavbosch@clinic.ub.es>
authors.forename: Xavier
authors.initials: X
authors.lastname: Bosch
authors.forename: Antonio
authors.initials: A
authors.lastname: Guilabert
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Eduard
authors.initials: E
authors.lastname: Mirapeix
mesh_terms: D000818:Animals; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology* / Q000378:metabolism; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000920:Antibody-Dependent Cell Cytotoxicity; D000921:Antibody-Producing Cells / Q000187:drug effects / Q000378:metabolism; D015551:Autoimmunity; D042783:Endothelial Cells / Q000276:immunology / Q000378:metabolism*; D006801:Humans; D018836:Inflammation Mediators / Q000276:immunology / Q000378:metabolism; D018375:Neutrophil Activation / Q000276:immunology*; D009504:Neutrophils / Q000276:immunology* / Q000378:metabolism; D019899:Paracrine Communication / Q000276:immunology; D016897:Respiratory Burst / Q000235:genetics / Q000276:immunology; D000069283:Rituximab; D015398:Signal Transduction / Q000276:immunology; D014657:Vasculitis / Q000276:immunology* / Q000503:physiopathology / Q000628:therapy*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D018836:Inflammation Mediators; D000069283:Rituximab