title: [Granulomatosis with polyangiitis].
abstract: Granulomatosis with polyangiitis (GPA) is a potentially life-threatening, rare disease. The etiology is unknown. GPA is histomorphologically characterized by extravascular necrotizing granulomatous inflammation and a systemic necrotizing vasculitis of small to medium-sized vessels. Clinically, a pulmonary-renal syndrome with pulmonary infiltrates, alveolar hemorrhage and a rapidly progressive glomerulonephritis is seen in about 80% of the cases with generalized disease. GPA is associated with proteinase 3-specific anti-neutrophil cytoplasmic autoantibodies (PR3-ANCA). Treatment is guided by severity of organ involvement and disease activity. Cytostatic immunosuppressants or the monoclonal anti-CD20 antibody rituximab are applied.
authors.forename: Anja
authors.initials: A
authors.lastname: Kerstein
authors.forename: Konstanze
authors.initials: K
authors.lastname: Holl-Ulrich
authors.forename: Antje
authors.initials: A
authors.lastname: Müller
authors.forename: Gabriela
authors.initials: G
authors.lastname: Riemekasten
authors.forename: Peter
authors.initials: P
authors.lastname: Lamprecht
mesh_terms: D003131:Combined Modality Therapy / Q000379:methods; D003937:Diagnosis, Differential; D019317:Evidence-Based Medicine; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D000069283:Rituximab / Q000008:administration & dosage*; D016896:Treatment Outcome
chemical_list: D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab