The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources.

OBJECTIVE To estimate the prevalence, annual mortality, and geographic distribution of Wegener's granulomatosis. METHODS Analysis of national vital statistics data and hospitalization data from a national survey and from all New York State inpatient facilities. RESULTS Between 1979 and 1988, 1,784 death certificates in the United States listed Wegener's granulomatosis as a cause of death. Nationally, an estimated 10,771 hospitalizations included Wegener's granulomatosis among the discharge diagnoses. In New York State, there were 978 hospitalizations among 571 individuals with Wegener's granulomatosis. CONCLUSION The prevalence of Wegener's granulomatosis in the United States is approximately 3.0 per 100,000 persons. Clear differences in the geographic distribution of Wegener's granulomatosis are apparent when analysis consider rates of disease in individual counties. Contrary to previous reports, associations between disease exacerbations and season were not apparent.