BIMODAL MORTALITY IN WEGENER'S GRANULOMATOSIS: RESULTS FROM THE UK GENERAL PRACTICE RESEARCH DATABASE

R. Luqmani, R. Suppiah, D. Culliford, C. Edwards, D. Jayne, J. Maskell, K. Morishita, R. Phillip, N. ArdenNuffield Orthopaedic Centre The University of Oxford, Oxford University of Southampton, Southampton Adenbrookes Hospital, Cambridge, United KingdomObjectives: To compare the long term mortality in patients with Wegener's granulomatosis (WG) compared to the general population. Methods: We used data from the General Practice Research Database which contains the computerized records of 6.25m patients and is representative of the population of the UK. We identified all subjects with a new diagnosis of WG in the period 1989 - 2004, and for each case, compared mortality with 10 controls matched for age, gender and practice. Results: We identified 255 patients with a new diagnosis of WG (mean age 58.1, range 9-90, 47% female) and 2546 controls (mean age 58.1, range 9-89, 47% female). Mean follow up was 6.4 years. The mortality for patients with WG was significantly increased during the first year [HR 9.0 (CI 5.8 -13.9)], especially for those ≤65 years of age [HR 19.9 (CI 8.8 - 44.9)]. The excess mortality was less marked after the first year: 1-5 years [HR 1.68 (CI 1.08 - 2.60)], 5-10 years [HR 2.41 (CI 1.43- 4.07)] and 10-15 years (HR 4.4 CI 2.0, 9.8). The Kaplan-Meier survival curve showed a sharp increase in mortality after 8 years. Conclusion: Despite current therapy, patients with WG have a 9-fold increased risk of death in the first year of disease. Between 5 and 8 years from diagnosis the risk is at its lowest, although remains higher than the background population. There is a rise in mortality after 8 years which currently remains unexplained. Disclosure of Interest: None declaredCitation: Annals of the Rheumatic Diseases, volume 69, supplement 3, year 2010, page 235Session: Vasculitis (Poster Presentations )