Survival of patients with Wegener's granulomatosis diagnosed in Finland in 1981-2000.

ABSTRACT

OBJECTIVE: To determine the survival of and contributing factors in patients diagnosed with Wegener's granulomatosis (WG) between 1981 and 2000 in Finland. METHODS: A retrospective cohort study using hospital discharge registers with a review of hospital case records. Analysis of causes of death in Finland up to 30 July 2005. RESULTS: A total of 492 patients received a verified diagnosis of WG between 1981 and 2000. Of these, 203 died before the end of June 2005. The overall 1-year survival rate was 83.3% and the 5-year survival rate was 74.2%. The standardized mortality ratio (SMR) for all WG patients was 3.43 [95% confidence interval (CI) 2.98-3.94], for women 4.38 (95% CI 3.59-5.61) and for men 2.80 (95% CI 2.28-3.41). The most frequent causes of death were WG or another connective tissue disease, cardiovascular events and neoplasms. The prognosis did not change markedly over the 20-year period. Older age and elevated creatinine level at presentation were associated with poorer prognosis, whereas primary ear, nose, and throat (ENT) involvement and prompt treatment with cyclophosphamide predicted longer survival. CONCLUSIONS: WG patients had increased mortality compared with the rest of the population. Older age and elevated creatinine level at diagnosis predicted poor prognosis, but ENT involvement initially and cyclophosphamide treatment resulted in a better outcome.