title: Treatment of severe and/or refractory ANCA-associated vasculitis.
abstract: Most patients presenting with systemic necrotizing vasculitides improve when they are adequately treated. The presence of life-threatening manifestations or visceral involvement modifying organ function characterizes severe vasculitis, confirmed by disease-severity scores. Sequelae cannot always be predicted and prevented but organ involvement present at disease onset requires rapid therapeutic intervention. Some patients present a persistent active disease, which does not respond to treatments and deserve other drugs or combination of drugs. The therapeutic options for severe and/or relapsing and refractory diseases are described.
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, INSERM U1060, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, University of Paris Descartes, 27, rue du faubourg Saint-Jacques, 75679, Paris Cedex 14, France, loic.guillevin@cch.aphp.fr.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000628:therapy*; D003603:Cytotoxins / Q000627:therapeutic use; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010951:Plasma Exchange / Q000379:methods
chemical_list: D003603:Cytotoxins; D005938:Glucocorticoids; D007166:Immunosuppressive Agents