title: Analysis of factors predictive of survival based on 49 patients with systemic Wegener's granulomatosis and prospective follow-up.
abstract: OBJECTIVE
  This prospective study attempted to determine factors predictive of survival in systemic Wegener's granulomatosis (WG) based on 49 patients. Patients and methods. All patients had previously untreated systemic WG. Treatment was with oral or pulse cyclophosphamide plus corticosteroids. Univariate and multivariate analyses of survival were performed using 13 parameters evaluated at diagnosis.
  
  
  RESULTS
  The mortality rate was 37% during a mean follow-up period of 1.9 yr. Among the 13 parameters evaluated, univariate analysis selected the following factors as predictors of a poor outcome: serum creatinine > or =18.1 mg/dl, age > or =57 yr, and erythrocyte sedimentation rate (ESR) > or =90 mm/1st h. The absence of ear, nose and throat (ENT) involvement also tended to predict a greater risk of mortality. Glomerulonephritis, when present and regardless of creatininaemia, and pulmonary involvement had no significant effect. Multivariate analysis retained serum creatinine > or =18.1 mg/dl and age > or =57 yr as significant predictors of poor prognosis.
  
  
  CONCLUSIONS
  Our results suggest that impaired renal function and older age are independent factors predicting poor outcome in WG. ESR proved to be a good marker of disease severity. Conversely, univariate analysis indicated that patients with ENT involvement tended to have a better outcome, suggesting a more benign evolution of granulomatous disease compared with more aggressive vasculitis.
authors.affiliation: Service de Médecine Interne, Hôpital Avicenne, Université Paris-Nord, 125, route de Stalingrad, 93009 Bobigny Cedex, France.
authors.forename: A
authors.initials: A
authors.lastname: Mahr
authors.forename: T
authors.initials: T
authors.lastname: Girard
authors.forename: R
authors.initials: R
authors.lastname: Agher
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000368:Aged; D002423:Cause of Death; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005602:France; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy / Q000401:mortality*; D006801:Humans; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D020551:Pulse Therapy, Drug; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D003520:Cyclophosphamide; D008775:Methylprednisolone