Objective
To estimate the incidence, prevalence and mortality of ANCA-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), in a U.S. based adult population.
Methods
All medical records of patients with a diagnosis or suspicion of AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. Incidence rates were age- and sex-adjusted to the 2010 US white population. Age- and sex-adjusted prevalence was calculated on January 1, 2015. Survival rates were compared with expected rates in the Minnesota population.
Results
Of 58 incident cases of AAV, 23 (40%) were GPA, 28 (48%) MPA, and 7 (12%) EGPA. Overall, 28 (48%) were women, 57 (98%) were Caucasian, with a mean±SD age at diagnosis of 61.1±16.5 years. Thirty-four patients (61%) were MPO-ANCA and 17 (30%) were PR3-ANCA positive; 5 (9%) were ANCA-negative.
The annual incidence of AAV was 3.3/100,000 population (95%CI: 2.4–4.1). GPA, MPA and EGPA incidence was 1.3 (95%CI: 0.8–1.8), 1.6 (95%CI: 1.0–2.2), and 0.4 (95%CI: 0.1–0.6), respectively. Overall prevalence of AAV was 42.1/100,000 (95%CI: 29.6–54.6). Mortality for AAV overall, and EGPA, MPA and MPO-ANCA was increased (p<0.05); mortality of GPA, PR3-ANCA and ANCA-negative patients did not differ from general population.
Conclusion
The annual incidence of AAV in Olmsted County is 3.3/100,000, with prevalence of 42.1/100,000, substantially higher than reported in other areas worldwide. The incidence of GPA and MPA is similar. Mortality of MPA and EGPA, but not GPA, is higher than the general population; MPO-ANCA is a marker of poor survival.