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Celiac Disease - Description
ICD-9
579.0, celiac disease
Other codes used include: 579.9 and
263.0. See Celiac ICD9
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Description
Celiac disease (CD) is a chronic genetic disease, the predominant feature of which is reversible small intestinal mucosal inflammation. This inflammation is triggered and perpetuated by dietary exposure to the gliadin peptide contained in gluten and found in wheat, barley, rye, and possibly oats. Skin involvement with dermatitis herpetiformis may also occur. The increased prevalence of type I diabetes mellitus, osteoporosis, intestinal lymphoma, autoimmune disease, miscarriage, and infertility in patients with celiac disease and their families suggests common immunogenetic etiologies. There is an increased prevalence of celiac disease in children with Down syndrome (see the Down syndrome and celiac disease Issue page). Treatment of celiac disease consists of complete elimination of gluten-containing foods from the diet and is monitored by clinical response (including growth response), serologic marker response, and small intestinal mucosa histologic response.Genetics
The genetics of celiac disease are not well understood. Genes confer a genetic susceptibility to celiac disease, but not all people with the same genes manifest the disease. Susceptibility to CD is associated with HLA genes; the majority of people with celiac disease have DQ2 or DQ8. First degree relatives of individuals with CD who themselves have symptoms of CD have a 1 in 22 risk of having CD themselves. [Fasano: 2003]Prognosis
Complete gluten restriction should result in complete cessation of the inflammatory response and subsequent normal reproductive capability and lifespan. However, complete gluten exclusion is difficult to maintain for a lifetime. Ongoing inflammation, even in the absence of symptoms, causes chronically increased metabolic demands, malabsorption of macro- and micro-nutrients, and increased cell proliferation, with risk of intestinal oncogenesis, particularly small bowel lymphoma.Prevalence
The prevalence of CD is estimated to be between 1:150 and 1:300, based on serologic antibody screening, and possibly as high as 1% in Caucasian populations. However prevalence estimates may vary depending on the clinical syndrome definition used in the study. [Hill: 2007] [Rodrigues: 2008] [Telega: 2008] [Garampazzi: 2007]Pearls And Alerts
On Initial Diagnosis Page
Helpful Articles
PubMed Search on Celiac Disease in children for last 3 years.
McGowan KE, Castiglione DA, Butzner JD.
The changing face of childhood celiac disease in north america: impact of serological testing.
Pediatrics.
2009;124(6):1572-8.
PubMed abstract
Hill ID.
Serologic testing for celiac disease: primum non nocere!.
J Pediatr.
2007;150(5):453-4.
PubMed abstract
Commentary regarding screening children with type 1 diabetes mellitus for celiac disease.
Hill ID, Dirks MH, Liptak GS, Colletti RB, Fasano A, Guandalini S, Hoffenberg EJ, Horvath K, Murray JA, Pivor M, Seidman EG.
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):1-19.
PubMed abstract / Full Text
Recommendations for the diagnosis and treatment of celiac disease in children from the North American Society for Pediatric
Gastroenterology, Hepatology, and Nutrition.
Green PH, Cellier C.
Celiac disease.
N Engl J Med.
2007;357(17):1731-43.
PubMed abstract
Excellent and extensive review article, not focused on children.
Celiac Disease Module Authors
| Authors: | Lynne M Kerr MD, PhD, 3/2009 Daniel Jackson M.D., 6/2008 |
| Content Last Updated: | 3/2009 |
The authors listed above are responsible for the overall Celiac Disease Module. Authors contributing to individual pages in the module are listed on those pages.
Page Bibliography
Fasano A, Berti I, Gerarduzzi T, Not T, Colletti RB, Drago S, Elitsur Y, Green PH, Guandalini S, Hill ID, Pietzak M, Ventura
A, Thorpe M, Kryszak D, Fornaroli F, Wasserman SS, Murray JA, Horvath K.
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(3):286-92.
PubMed abstract
Garampazzi A, Rapa A, Mura S, Capelli A, Valori A, Boldorini R, Oderda G.
Clinical pattern of celiac disease is still changing.
J Pediatr Gastroenterol Nutr.
2007;45(5):611-4.
PubMed abstract
Hill ID.
Serologic testing for celiac disease: primum non nocere!.
J Pediatr.
2007;150(5):453-4.
PubMed abstract
Commentary regarding screening children with type 1 diabetes mellitus for celiac disease.
Rodrigues AF, Jenkins HR.
Investigation and management of coeliac disease.
Arch Dis Child.
2008;:251-4.
PubMed abstract
Telega G, Bennet TR, Werlin S.
Emerging new clinical patterns in the presentation of celiac disease.
Arch Pediatr Adolesc Med.
2008;162(2):164-8.
PubMed abstract