The Epidemiology of Antineutrophil Cytoplasmic Autoantibody–Associated Vasculitis in Olmsted County, Minnesota

Abstract

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.

Risk of Cardiovascular Disease and Venous Thromboembolism Among Patients With Incident ANCA-Associated Vasculitis: A 20-Year Population-Based Cohort Study

“…Incident cases of AAV from January 1, 1996 to December 31, 2015 this geographically defined region of the U.S. were previously identified by medical record review (6). AAV cases were included if they fulfilled the criteria of at least one of the following classification schemes: American College of Rheumatology (ACR) classification criteria or the modified ACR criteria for GPA (26) (27), ACR criteria for EGPA (28), Chapel Hill Consensus Conference (CHCC) definition (29), or European Medical Agency (EMA) algorithm (30).…”

“…Among AAV, granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis) and microscopic polyangiitis (MPA) are the two most common subtypes, and with eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) account for an estimated combined prevalence of 42.1 per 100,000 adult population in the United States (U.S.) (6). Modern treatments have greatly improved survival in AAV (7–10), but many patients suffer cardiovascular and thrombotic complications during long-term follow up (11, 12).…”

Perinuclear anti neutrophil cytoplasmic antibody vasculitis with nonspecific muscle pain

“…As per recent study, ANCA associated vasculitis have annual incidence ranging from 1.2 to 2.0 cases per 1,00,000 individuals and a prevalence of 4.6-18.4 cases per 1,00,000 individuals. 2 The term "polyangiitis" is preferred to "polyarteritis" for Microscopic Polyangitis because of the tendency of the disease to involve veins as well as arteries. The Chapel Hill Consensus Conference (2012) defined Microscopic Polyangitis as a process that i) involves necrotizing vasculitis with few or no immune deposits; ii) affects small blood vessels (capillaries, arterioles, or venules) and possibly medium-sized vessels, as well; and iii) demonstrates a tropism for the kidneys and lungs.…”

Vasculitis

“…The frequency of various types of vasculitis is summarized in Table 7.3 [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]. While Wegener granulomatosis is more common in the Northern Hemisphere, polyarteritis nodosa and microscopic polyangiitis are more common in Southern Europe and especially in Arab countries.…”