title: [Granulomatosis with polyangiitis (Wegener). Description of 15 cases].
abstract: BACKGROUND
  Granulomatosis with polyangiitis (GP) is a necrotizing vasculitis of unknown etiology that involves small and medium caliber vessels. It is associated with anti neutrophil cytoplasm antibodies (ANCA). It most often affects the respiratory tract and the kidneys and its most important pathologic feature is the presence of necrotizing granulomas.
  
  
  OBJECTIVE
  To detail the features of 15 patients with GP diagnosed in a university referral center.
  
  
  METHODS
  Retrospective study: between 1984 and 2009, 15 patients with GP were diagnosed in our center. Epidemiological, clinical, laboratory test as well as pathologic studies and treatment were retrospectively analyzed. Biopsy diagnosis of GP was considered as an inclusion criterion.
  
  
  RESULTS
  Fifteen patients were diagnosed: 12 men and 3 women. Mean age at diagnosis: 52.2 years (14-78). 12 patients had a history of smoking. A biopsy was diagnostic in all patients. ANCA were positive in 11 cases, 6 had a cytoplasmic c-ANCA pattern. All patients had pulmonary involvement and seven (40%) had renal involvement. All patients received intravenous glucocorticoids and cyclophosphamide as induction therapy. During the disease progression 5 patients died.
  
  
  CONCLUSIONS
  The clinical features of this series do not differ from those described by other authors. However, a history of smoking is more common than expected. Frequently used drugs were glucocorticoids and cyclophosphamide (oral and pulse therapy). The course was usually unfavorable, with outbreaks or complications due to immunosuppression, except for those with limited forms. Immunosuppressive therapy should be maintained indefinitely in most cases.
authors.affiliation: Sección de Reumatología, Hospital Universitario Germans Trias i Pujol, Barcelona, España. melaniamm@gmail.com
authors.forename: Melania
authors.initials: M
authors.lastname: Martínez-Morillo
authors.forename: Dolors
authors.initials: D
authors.lastname: Grados
authors.forename: Dolores
authors.initials: D
authors.lastname: Naranjo-Hans
authors.forename: Lourdes
authors.initials: L
authors.lastname: Mateo
authors.forename: Susana
authors.initials: S
authors.lastname: Holgado
authors.forename: Alejandro
authors.initials: A
authors.lastname: Olivé
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult