title: Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades.
abstract: OBJECTIVE
  To determine the long-term outcome in patients with Wegener's granulomatosis (WG) over 4 decades in an academic hospital unit specializing in rheumatology.
  
  
  METHODS
  We included 290 patients, divided them into 2 cohorts, and compared them with the historical cohort of 155 patients. Comparisons were retrospective regarding disease manifestations, therapy, mortality, and incidence of malignancies. The historical cohort (cohort 1) included 155 patients diagnosed between 1966 and 1993, cohort 2 included 123 patients diagnosed between 1994 and 1998, and cohort 3 included 167 patients diagnosed between 1999 and 2002.
  
  
  RESULTS
  Over time, the interval between first symptoms and diagnosis was reduced by half (from 8 months to 4 months). Organ manifestations were similar in the 3 cohorts, and more than 80% of patients still required cyclophosphamide (CYC); however, the median cumulative dose was reduced significantly (from 67 gm in cohort 1 to 36 gm in cohort 2 and to 24 gm in cohort 3). The standardized mortality ratios (SMRs) declined (from 2.1 in cohort 1 to 1.41 in cohort 2 and to 1.03 in cohort 3), with fewer deaths related to WG and/or therapy (86.4% in cohort 1, 76.9% in cohort 2, 50% in cohort 3), decreasing relapse rates (63.9% in cohort 1, 51.2% in cohort 2, 35.3% in cohort 3), and no increased rate of malignancies. Compared with young females, young males had a considerably higher SMR (8.87 [95% confidence interval 4.05-16.8]) and more frequent renal manifestations (54.4% versus 33.8%).
  
  
  CONCLUSIONS
  Mortality of WG patients declined over the last 4 decades, probably due to improved diagnostic and therapeutic procedures and increased awareness of WG, which led to earlier diagnosis and therapy, reduction in relapse rates, and lower cumulative CYC dose with fewer deaths related to therapy.
authors.affiliation: University Hospital Schleswig-Holstein Campus Lübeck, Lübeck, Germany.
authors.forename: Julia U
authors.initials: JU
authors.lastname: Holle
authors.forename: Wolfgang L
authors.initials: WL
authors.lastname: Gross
authors.forename: Ute
authors.initials: U
authors.lastname: Latza
authors.forename: Bernhard
authors.initials: B
authors.lastname: Nölle
authors.forename: Petra
authors.initials: P
authors.lastname: Ambrosch
authors.forename: Martin
authors.initials: M
authors.lastname: Heller
authors.forename: Regina
authors.initials: R
authors.lastname: Fertmann
authors.forename: Eva
authors.initials: E
authors.lastname: Reinhold-Keller
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018501:Antirheumatic Agents / Q000627:therapeutic use; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005858:Germany; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D010353:Patient Education as Topic; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; D005938:Glucocorticoids; D003520:Cyclophosphamide; D011239:Prednisolone; D008727:Methotrexate