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ANCA-Associated Vasculitis (AAV)

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Epidemiology

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare autoimmune conditions characterized by inflammation of the blood vessels, especially the small blood vessels. Blood vessel inflammation adversely affects multiple organ systems.1,2 The 3 primary forms of AAV are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Additional ANCA-associated conditions include drug-induced vasculitis and renal-limited vasculitis.1

Incidence and Prevalence of AAV

Each type of AAV is a rare condition. The incidence is estimated to be between 10 and 20 individuals per million.1 The prevalence is estimated to be between 200 and 400 individuals per million. Because of advances in diagnostic testing and the identification and classification of disease, AAV is becoming increasingly recognized and diagnosed, with an accompanying increase in the annual incidence.3

Read more about AAV diagnosis

Geographical Incidence and Prevalence of AAV

Epidemiology Studies on AAV in Europe

A 10-year study of primary systemic vasculitides, including ANCA-associated conditions, was conducted from 1988 to 1998 in the United Kingdom. In this study, the annual incidence was 19.8 cases per million and the point prevalence was 144.5 cases per million.4 

According to a study published in 2003, the annual incidence of primary systemic vasculitides in northwest Spain was approximately 13.07 cases per million.5

In southern Sweden, researchers separated the annual incidence rates of primary AAV by disease subtype. According to their study, published in 2009, the annual incidence of GPA was approximately 9.8 cases per million, the incidence of MPA was 10.1 cases per million, and incidence of EGPA was approximately 0.9 cases per million. The 1- and 5-year survival rates for all patients were 87.8% and 71.6%, respectively. Survival rates were lower in patients with a diagnosis of MPA than in those with GPA.6

An earlier study, also conducted in Sweden and published in 2007, reported the point prevalence of GPA to be 160 cases per million, that of MPA to be 94 cases per million, and that of EGPA to be 14 cases per million.7

In northeastern France, annual incidence rates of MPA, GPA, and EGPA were 25.1, 23.7, and 10.7 cases per million, respectively, according to a study published in 2000.8

When different regions throughout Europe were compared, GPA was more prevalent in the north, and MPA was more prevalent in the south, although the overall incidence rates of all AAV conditions were similar across 3 regions of Europe, at approximately 19 cases per million.9,10

Read more about AAV etiology

Epidemiology Studies on AAV in Australia and New Zealand

In Australia, annual incidence rates of GPA, MPA, and EGPA between 1995 and 1999 were 8.8, 2.3, and 2.3 cases per million, respectively. Between 2000 and 2004, the annual incidence rates of GPA, MPA, and EGPA were 8.4, 5.0, and 2.2 cases per million, respectively. The period prevalence rates for all conditions in these 2 time periods were 95 cases per million and 148 cases per million, respectively. The researchers noted that GPA and MPA occurred more frequently in rural locations than in urban locations. GPA also occurred more frequently in southeastern Australia than in southern Europe.11

In New Zealand, the 5-year period prevalence of GPA between 1999 and 2003 was 152 cases per million, and that of MPA was 58 cases per million. Point prevalence during this time period for GPA was 112 cases per million, and for MPA it was 37 cases per million.12

Epidemiology Studies on AAV in Asia

In Japan, annual incidence rates of GPA and MPA between 2005 and 2009 were 2.1 and 18.2 cases per million, respectively. The period prevalence rates in 2002 for GPA, MPA, and EGPA were 2.3, 13.8, and 1.0 cases per million, respectively. The period prevalence rate in 2009 for EGPA was 17.8 cases per million.9

In Taiwan, the annual incidence rate of GPA between 1997 and 2008 was 0.37 cases per million.9 

Epidemiology Studies on AAV in North America

In the United States, a 20-year population-based study conducted in Minnesota between January 1996 and December 2015 reported the annual incidence of AAV to be 3.3 cases per 100,000. The annual incidence according to disease subtype was 1.3 cases per 100,000 for GPA, 1.6 cases per 100,000 for MPA, and 0.4 cases per 100,000 for EGPA.13

Read more about AAV complications

Sex Factors of AAV

Some sources report equal prevalence in males and females,3 whereas others suggest a slight male predominance.2,4,5,13 In analyzing disease subtypes, some sources report a male-to-female ratio of 2:1 among patients with MPA.1

Read more about AAV signs and symptoms

Race/Ethnicity Factors of AAV

Sources suggest that a genetic predisposition to AAV is higher among Europeans.8 

A mixed ethnicity study conducted in the United Kingdom between March 2007 and June 2013 reported that AAV occurred more frequently among White individuals (incidence of 25.8 per million person-years) than among Black individuals and persons of other ethnic groups (incidence of 8.4 per million person-years). However, the difference between ethnic groups did not reach statistical significance (P =.3).14

Another study revealed that Black individuals with confirmed PR3-ANCA vasculitis had a 73.3-fold higher likelihood than controls of carrying HLA-DRB1*15 alleles, and White individuals had a 2.2-fold higher likelihood than controls of carrying DRB1*1501 alleles.15

Read more about AAV genetics

Age Factors of AAV

The incidence of GPA peaks during later middle age, from age 55 to age 60.1,2 However, AAV does occur in younger patients, and relapses were more frequent in children with GPA. In adolescent patients, AAV showed a female predominance.16

Read more about AAV risk factors

Impact of Disease Subtype on AAV

Of the 3 disease subtypes, GPA occurs most frequently, followed closely by MPA, which occurs at a 2:1 male-to-female ratio. EGPA is the least common of the 3 primary ANCA-associated conditions.1

Read more about AAV types

References

  1. Qasim A, Patel JB. ANCA positive vasculitis. StatPearls [Internet]. Updated May 29, 2022. Accessed March 3, 2023.
  2. Yates M, Watts R. ANCA-associated vasculitis. Clin Med (Lond). 2017;17(1):60-64. doi:10.7861/clinmedicine.17-1-60
  3. Almaani S, Fussner LA, Brodsky S, Meara AS, Jayne D. ANCA-associated vasculitis: an update. J Clin Med. 2021;10(7):1446. doi:10.3390/jcm10071446
  4. Watts RA, Lane SE, Bentham G, Scott DGI. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. 2000;43(2):414-419. doi:10.1002/1529-0131(200002)43:2<414::AID-ANR23>3.0.CO;2-0
  5. Gonzalez-Gay MA, Garcia-Porrua C, Guerrero J, Rodriguez-Ledo P, Llorca J. The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions. Arthritis Rheum. 2003;49(3):388-393. doi:10.1002/art.11115
  6. Mohammad AJ, Jacobsson LTH, Westman KWA, Sturfelt G, Segelmark M. Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome and polyarteritis nodosa. Rheumatology. 2009;48(12):1560-1565. doi:10.1093/rheumatology/kep304
  7. Mohammad AJ, Jacobsson LTH, Mahr AD, Sturfelt G, Segelmark M. Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg–Strauss syndrome within a defined population in southern Sweden. Rheumatology. 2007;46(8):1329-1337. doi:10.1093/rheumatology/kem107
  8. Mahr A, Guillevin L, Poissonnet M, Aymé S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51(1):92-99. doi:10.1002/art.20077
  9. Watts RA, Mahr A, Mohammad AJ, Gatenby P, Basu N, Flores-Suárez LF. Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Nephrol Dial Transplant. 2015;30 Suppl 1:i14-i22. doi:10.1093/ndt/gfv022
  10. Watts R, Lane S, Scott D, et al. Epidemiology of vasculitis in Europe. Ann Rheum Dis. 2001;60(12):1156-1157. doi:10.1136/ard.60.12.1156a
  11. Ormerod AS, Cook MC. Epidemiology of primary systemic vasculitis in the Australian Capital Territory and south-eastern New South Wales. Intern Med J. 2008;38(11):816-823. doi:10.1111/j.1445-5994.2008.01672.x
  12. Gibson A, Stamp LK, Chapman PT, O’Donnell JL. The epidemiology of Wegener’s granulomatosis and microscopic polyangiitis in a Southern Hemisphere region. Rheumatology. 2006;45(5):624-628. doi:10.1093/rheumatology/kei259
  13. Berti A, Cornec D, Crowson CS, Specks U, Matteson EL. The epidemiology of ANCA associated vasculitis in Olmsted County, Minnesota (USA): a 20 year population-based study. Arthritis Rheumatol. 2017;69(12):2338-2350. doi:10.1002/art.40313
  14. Pearce FA, Lanyon PC, Grainge MJ, et al. Incidence of ANCA-associated vasculitis in a UK mixed ethnicity population. Rheumatology. 2016;55(9):1656-1663. doi:10.1093/rheumatology/kew232
  15. Cao Y, Schmitz JL, Yang J, et al. DRB1*15 allele is a risk factor for PR3-ANCA disease in African Americans. J Am Soc Nephrol. 2011;22(6):1161-1167. doi:10.1681/ASN.2010101058
  16. Iudici M, Quartier P, Terrier B, Mouthon L, Guillevin L, Puéchal X. Childhood-onset granulomatosis with polyangiitis and microscopic polyangiitis: systematic review and meta-analysis. Orphanet J Rare Dis. 2016;11:141. doi:10.1186/s13023-016-0523-y

Reviewed by Debjyoti Talukdar, MD, on 3/14/2023.

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