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Prevalence of celiac disease in special populations

Prevalence of celiac disease in special populations
  Percent of group affected Fold increase in risk compared with general population
General population (United States and Europe)[1] 0.7 to 1.0 –
Relatives of patient with celiac disease*[1,2]
First-degree relatives 5 to 7.5 5 to 11
Second-degree relatives 2 to 3 2 to 4
Down syndrome[3,4] 5 to 12 7 to 18
Type 1 diabetes[3,9] 5 to 10 7 to 13
Autoimmune liver disease[5] 3.6 6.6
Juvenile idiopathic arthritis[6-8] 2 to 3 3
IgA deficiency[10] 8 11
Autoimmune thyroid disease[3,11] 2 to 7 6 (less in children)
Turner syndrome[3] 4 to 8 5 to 11
Williams syndrome[12] 8 11

IgA: immunoglobulin A.

* The risk also varies with the relationship to the proband (eg, higher for siblings than for parents of the proband)[2].
Data from:
  1. Fasano A, Berti I, Gerarduzzi T, et al. Prevalence of celiac disease in at-risk and not-at-risk groups in the United States: A large multicenter study. Arch Intern Med 2003; 163:286.
  2. Singh P, Arora S, Lal S, et al. Risk of celiac disease in the first- and second-degree relatives of patients with celiac disease: A Systematic Review and Meta-Analysis. Am J Gastroenterol 2015; 110:1539.
  3. Hill ID, Dirks MH, Liptak GS, et al. Guideline for the diagnosis and treatment of celiac disease in children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 2005; 40:1.
  4. Rostom A, Murray JA, Kagnoff MF. American Gastroenterological Association (AGA) Institute technical review on the diagnosis and management of celiac disease. Gastroenterology 2006; 131:1981.
  5. Vajro P, Paolella G, Maggiore G, Giordano G. Pediatric celiac disease, cryptogenic hypertransaminasemia, and autoimmune hepatitis. J Pediatr Gastroenterol Nutr 2013; 56:663.
  6. Sherman Y, Karanicolas R, DiMarco B, et al. Unrecognized celiac disease in children presenting for rheumatology evaluation. Pediatrics 2015; 136:e68.
  7. Poddighe D, Romano M, Dossybayeva K, et al. Celiac disease in juvenile idiopathic arthritis and other pediatric rheumatic disorders. J Clin Med 2022; 11:1089.
  8. Doyle JB, Lebwohl B, Askling J, et al. Risk of juvenile idiopathic arthritis and rheumatoid arthritis in patients with celiac disease: A population-based cohort study. Am J Gastroenterol 2022; 117:1971.
  9. Crone J, Rami B, Huber WD, et al. Prevalence of celiac disease and follow-up of EMA in children and adolescents with type 1 diabetes mellitus. J Pediatr Gastroenterol Nutr 2003; 37:67.
  10. Meini A, Pillan NM, Villanacci V, et al. Prevalence and diagnosis of celiac disease in IgA-deficient children. Ann Allergy Asthma Immunol 1996; 77:333.
  11. Ch'ng CL, Biswas M, Benton A, et al. Prospective screening for coeliac disease in patients with Graves' hyperthyroidism using anti-gliadin and tissue transglutaminase antibodies. Clin Endocrinol (Oxf) 2005; 62:303.
  12. Giannotti A, Tiberio G, Castro M, et al. Coeliac disease in Williams syndrome. J Med Genet 2001; 38:767.
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