title: [Granulomatosis with polyangiitis - clinical picture and review of current treatment options].
abstract: Granulomatosis with polyangiitis (GPA) is a rare disease of unknown aetiology, characterized by necrotizing inflammation of small and medium-sized vessels and formation of granulomas. The disease typically involves the upper and lower respiratory tracts and the kidneys, but it can occur in many other locations, often giving a surprising clinical picture. The majority of patients have autoantibodies against cytoplasm of neutrophils (ANCA), of which a pathogenetic role is postulated. The disease affects mostly middle-aged and elderly people, with a predominance of Caucasians. Before the era of immunosuppressive therapy GPA was associated with high early mortality. Nowadays, we have a treatment that gives remission in the majority of patients. The decision about the method of treatment should be made depending on the clinical form of the disease (local or generalized, refractory). Standard remission induction therapy in the generalized disease is cyclophosphamide in combination with steroids. This therapy, however effective, is associated with a number of side effects. In addition, some patients are resistant to standard therapy, and half of them experience relapses. Therefore, there is need for further research for alternative treatment and maintenance therapy. Currently, hopes are raised by biological agents. Among them, the best studied so far is rituximab, which found a place in the current recommendations of EULAR as an alternative drug for patients with refractory disease. The article discusses the clinical picture of GPA, diagnosis and currently recommended treatment.
authors.affiliation: Klinika Pulmonologii, Alergologii i Onkologii Pulmonologicznej Uniwersytetu Medycznego im. Karola Marcinkowskiego w Poznaniu. marta.lembicz7@wp.pl.
authors.forename: Marta
authors.initials: M
authors.lastname: Lembicz
authors.forename: Halina
authors.initials: H
authors.lastname: Batura-Gabryel
authors.forename: Agata
authors.initials: A
authors.lastname: Nowicka
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab