title: [Update on granulomatosis with polyangitis (GPA, Wegener's granulomatosis)].
abstract: Granulomatosis with polyangitis (GPA, Wegener's granulomatosis) is characterized by a granulomatous inflammation of the respiratory tract and a necrotizing ANCA-associated small to medium-size vessel vasculitis with a predilection for the lungs (pulmonary capillaritis) and kidneys (necrotizing glomerulonephritis). The disease evolves stage-wise and typically starts as inflammation of the respiratory tract followed by development of systemic vasculitis manifestations. Today, treatment is evidence-based and adapted according to activity and disease stage which has resulted in a significant improvement in long-term outcome. Early mortality during the first year of treatment poses one of the main problems and is a result of infections under immunosuppressive treatment. Furthermore, treatment of refractory disease activity which is often represented by granulomatous manifestations is still a challenge and may result in significant organ damage if not treated successfully.
authors.affiliation: UKSH, Campus Lübeck, Poliklinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt, Oskar-Alexander Str. 26, 24576, Bad Bramstedt, Deutschland. j.holle@klinikumbb.de
authors.forename: J U
authors.initials: JU
authors.lastname: Holle
authors.forename: E
authors.initials: E
authors.lastname: Reinhold-Keller
authors.forename: W L
authors.initials: WL
authors.lastname: Gross
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents