HLA-B27 is present in about 90% of patients who have AS.[18]Chatzikyriakidou A, Voulgari PV, Drosos AA. What is the role of HLA-B27 in spondyloarthropathies? Autoimmun Rev. 2011 Jun;10(8):464-8.
http://www.ncbi.nlm.nih.gov/pubmed/21296192?tool=bestpractice.com
The prevalence of HLA-B27 varies among ethnic communities in the US, with 7.5% of non-Hispanic white, 4.6% of Mexican-American, and 1.1% of non-Hispanic black people being HLA-B27 positive.[19]Reveille JD, Hirsch R, Dillon CF, et al. The prevalence of HLA-B27 in the US: data from the US National Health and Nutrition Examination Survey, 2009. Arthritis Rheum. 2012 May;64(5):1407-11.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4038331
http://www.ncbi.nlm.nih.gov/pubmed/22139851?tool=bestpractice.com
HLA-B27 contribution to the total genetic risk for ankylosing spondylitis (AS) is, however, relatively modest (approximately 20%).[20]Hanson A, Brown MA. Genetics and the causes of ankylosing spondylitis. Rheum Dis Clin North Am. 2017 Aug;43(3):401-14.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6982368
http://www.ncbi.nlm.nih.gov/pubmed/28711142?tool=bestpractice.com
[21]Ellinghaus D, Jostins L, Spain SL, et al. Analysis of five chronic inflammatory diseases identifies 27 new associations and highlights disease-specific patterns at shared loci. Nat Genet. 2016 May;48(5):510-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4848113
http://www.ncbi.nlm.nih.gov/pubmed/26974007?tool=bestpractice.com
Global prevalence of AS ranges from 9 to 30 people per 10,000 in the general population.[22]Wang R, Ward MM. Epidemiology of axial spondyloarthritis: an update. Curr Opin Rheumatol. 2018 Mar;30(2):137-43.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5811203
http://www.ncbi.nlm.nih.gov/pubmed/29227352?tool=bestpractice.com
The mean AS prevalence per 10,000 people (from 36 eligible studies) has been estimated as 23.8 in Europe, 16.7 in Asia, 31.9 in North America, and 10.2 in Latin America. Additional estimates, weighted by study size, were calculated as 18.6, 18.0, and 12.2 for Europe, Asia, and Latin America, respectively.[23]Dean LE, Jones GT, MacDonald AG, et al. Global prevalence of ankylosing spondylitis. Rheumatology (Oxford). 2014 Apr;53(4):650-7.
http://rheumatology.oxfordjournals.org/content/53/4/650.long
http://www.ncbi.nlm.nih.gov/pubmed/24324212?tool=bestpractice.com
Survey data (2009-2010) suggest an overall age-adjusted prevalence of definite and probable spondylarthritis of 0.9% in the US.[24]Reveille JD, Witter JP, Weisman MH. Prevalence of axial spondylarthritis in the United States: estimates from a cross-sectional survey. Arthritis Care Res (Hoboken). 2012 Jun;64(6):905-10.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4032290
http://www.ncbi.nlm.nih.gov/pubmed/22275150?tool=bestpractice.com
Low prevalence rates of AS have been reported in Japan and Greece.[25]Hukuda S, Minami M, Saito T, et al. Spondyloarthropathies in Japan: nationwide questionnaire survey performed by the Japan Ankylosing Spondylitis Society. J Rheumatol. 2001 Mar;28(3):554-9.
http://www.ncbi.nlm.nih.gov/pubmed/11296958?tool=bestpractice.com
[26]Alamanos Y, Papadopoulos NG, Voulgari PV, et al. Epidemiology of ankylosing spondylitis in Northwest Greece, 1983-2002. Rheumatology (Oxford). 2004 May;43(5):615-8.
http://rheumatology.oxfordjournals.org/content/43/5/615.full
http://www.ncbi.nlm.nih.gov/pubmed/14872102?tool=bestpractice.com
Prevalence rates of 0.37%, 0.3%, and 0.86% have been reported in Italy, France, and Germany, respectively.[27]De Angelis R, Salaffi F, Grassi W. Prevalence of spondyloarthropathies in an Italian population sample: a regional community-based study. Scand J Rheumatol. 2007 Jan-Feb;36(1):14-21.
http://www.ncbi.nlm.nih.gov/pubmed/17454930?tool=bestpractice.com
[28]Saraux A, Guillemin F, Guggenbuhl P, et al. Prevalence of spondyloarthropathies in France: 2001. Ann Rheum Dis. 2005 Oct;64(10):1431-5.
https://ard.bmj.com/content/64/10/1431.long
http://www.ncbi.nlm.nih.gov/pubmed/15817661?tool=bestpractice.com
[29]Braun J, Bollow M, Remlinger G, et al. Prevalence of spondylarthropathies in HLA-B27 positive and negative blood donors. Arthritis Rheum. 1998 Jan;41(1):58-67.
http://www.ncbi.nlm.nih.gov/pubmed/9433870?tool=bestpractice.com
Incidence rates vary between 0.4 per 100,000 in Iceland to 15 per 100,000 in Canada.[30]Bohn R, Cooney M, Deodhar A, et al. Incidence and prevalence of axial spondyloarthritis: methodologic challenges and gaps in the literature. Clin Exp Rheumatol. 2018 Mar-Apr;36(2):263-74.
https://www.clinexprheumatol.org/abstract.asp?a=11841
http://www.ncbi.nlm.nih.gov/pubmed/29148402?tool=bestpractice.com
In the US, an annual incidence rate of 7.3 per 100,000 person-years has been reported.[31]Carbone LD, Cooper C, Michet CJ, et al. Ankylosing spondylitis in Rochester, Minnesota, 1935-1989. Is the epidemiology changing? Arthritis Rheum. 1992 Dec;35(12):1476-82.
http://www.ncbi.nlm.nih.gov/pubmed/1472124?tool=bestpractice.com
AS commonly presents in the second decade of life.[32]Feldtkeller E, Bruckel J, Khan MA. Scientific contributions of ankylosing spondylitis patient advocacy groups. Curr Opin Rheumatol. 2000 Jul;12(4):239-47.
http://www.ncbi.nlm.nih.gov/pubmed/10910174?tool=bestpractice.com
Men are more frequently affected than women (2.5:1).[33]Kennedy LG, Will R, Calin A. Sex ratio in the spondyloarthropathies and its relationship to phenotypic expression, mode of inheritance and age at onset. J Rheumatol. 1993 Nov;20(11):1900-4.
http://www.ncbi.nlm.nih.gov/pubmed/8308776?tool=bestpractice.com
[34]Will R, Edmunds L, Elswood J, et al. Is there sexual inequality in ankylosing spondylitis? A study of 498 women and 1202 men. J Rheumatol. 1990 Dec;17(12):1649-52.
http://www.ncbi.nlm.nih.gov/pubmed/2084239?tool=bestpractice.com
[35]Moll JM, Wright V. New York clinical criteria for ankylosing spondylitis. A statistical evaluation. Ann Rheum Dis. 1973 Jul;32(4):354-63.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1006116/pdf/annrheumd00016-0073.pdf
http://www.ncbi.nlm.nih.gov/pubmed/4269429?tool=bestpractice.com
Early use of magnetic resonance imaging has resulted in increased detection of AS, and reduced male-to-female prevalence (1.21 prevalence ratio) has been reported in one study.[36]Haroon NN, Paterson JM, Li P, et al. Increasing proportion of female patients with ankylosing spondylitis: a population-based study of trends in the incidence and prevalence of AS. BMJ Open. 2014 Dec 15;4(12):e006634.
https://bmjopen.bmj.com/content/4/12/e006634.long
http://www.ncbi.nlm.nih.gov/pubmed/25510888?tool=bestpractice.com
There is a delay in diagnosis of about 8 years between symptom-onset and diagnosis, often leading to worse clinical outcomes.[16]Stone M, Warren RW, Bruckel J, et al. Juvenile-onset ankylosing spondylitis is associated with worse functional outcomes than adult-onset ankylosing spondylitis. Arthritis Rheum. 2005 Jun 15;53(3):445-51.
http://onlinelibrary.wiley.com/doi/10.1002/art.21174/full
http://www.ncbi.nlm.nih.gov/pubmed/15934110?tool=bestpractice.com
[37]Braun J, Sieper J. Inception cohorts for spondyloarthropathies [in German]. Z Rheumatol. 2000 Apr;59(2):117-21.
http://www.ncbi.nlm.nih.gov/pubmed/10868019?tool=bestpractice.com
Juvenile-onset AS has greater peripheral joint involvement and is associated with a worse clinical outcome.[16]Stone M, Warren RW, Bruckel J, et al. Juvenile-onset ankylosing spondylitis is associated with worse functional outcomes than adult-onset ankylosing spondylitis. Arthritis Rheum. 2005 Jun 15;53(3):445-51.
http://onlinelibrary.wiley.com/doi/10.1002/art.21174/full
http://www.ncbi.nlm.nih.gov/pubmed/15934110?tool=bestpractice.com
[37]Braun J, Sieper J. Inception cohorts for spondyloarthropathies [in German]. Z Rheumatol. 2000 Apr;59(2):117-21.
http://www.ncbi.nlm.nih.gov/pubmed/10868019?tool=bestpractice.com
Approximately 9% to 21% of AS cases in white populations have a juvenile onset.[16]Stone M, Warren RW, Bruckel J, et al. Juvenile-onset ankylosing spondylitis is associated with worse functional outcomes than adult-onset ankylosing spondylitis. Arthritis Rheum. 2005 Jun 15;53(3):445-51.
http://onlinelibrary.wiley.com/doi/10.1002/art.21174/full
http://www.ncbi.nlm.nih.gov/pubmed/15934110?tool=bestpractice.com