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Celiacs Helping Celiacs

Celiac Disease Defined

What is Celiac Disease?

Celiac disease (CD) is a genetic disorder. In people with CD, eating certain types of protein, called gluten, sets off an autoimmune response that causes damage to the small intestine. This, in turn, causes the small intestine to lose its ability to absorb the nutrients found in food, leading to malnutrition and a variety of other complications.

The offending protein, gluten, is found in wheat, barley, rye, and to a lesser extent, oats (WBRO). Related proteins are found in triticale, spelt, kamut. Refer to grains and flours Glossary for a more extensive list of both safe and offending grains.

 

Celiac Disease is:

  • a genetic, inheritable disease.
  • linked to genetically transmitted histocompatibility cell antigens (HLA DR3-DQ2, DR5/7 DQ2, and DR4-DQ8).
  • COMMON. Approximately 1 in 133 people have CD, however, only about 3% of these have been diagnosed. This means that there are over 2.1 million undiagnosed people with celiac disease in the United States.
  • characterized by damage to the mucosal lining of the small intestine which is known as villous atrophy.
  • responsible for the malabsorption of nutrients resulting in malnutrition.
  • linked to skin blisters known as dermatitis herpetiformis (DH).
  • not age-dependent. It may become active at any age.

Celiac Disease is NOT:

   

  • simply a food allergy.
  • an idiosyncratic reaction to food proteins (mediated by IgE).
  • typified by a rapid histamine-type reaction (such as bronchospasm, urticaria, etc.).

The Damaging Proteins

The term "gluten" is, in a sense, a generic term for the storage proteins that are found in grains. In reality, each type of protein - gliadin in wheat, secalin in rye, hordein in barley, avenin in oats, zein in corn and oryzenin in rice - is slightly different from the others. The "gluten" in wheat, rye, barley, and in a much lower amount, oats, contains particular amino acid sequences that are harmful to persons with celiac disease. The damaging proteins are particularly rich in proline and glutamine (especially the amino acid sequences which are in the following orders: Pro-Ser-Gln-Gln and Gln-Gln-Gln-Pro). As peptides, some such as 33-MER, cannot be broken down any further. In people with celiac disease, 33-MER stimulates T-cells to produce antibodies. The antibodies, in turn, attack the villi in the small intestine, reducing their ability to absorb nutrients. It is important to note that these sequences are NOT found in the proteins of corn and rice.

The Nature of the Injury

The damage to the small intestine (the jejunum) caused by this disease is very slow to develop and is insidious. It is:

  • almost certainly mediated by the immune system.
  • associated with ANTIBODIES to gliadin, reticulin and/or endomysial (smooth muscle) proteins.
  • probably not directly caused by the antibodies, though they may be signals for cell-mediated immunity.
  • probably produced by the cellular immune system (T cells) - but only when gluten-type prolamins are present.
  • reversible, in most cases, to completely normal bowel function, if the injurious protein is excluded from the diet.

How Does One "Catch" Celiac Disease?

Celiac disease cannot be "caught," but rather the potential for CD may be in the body from birth. Its onset is not confined to a particular age range or gender, although more women are diagnosed than men. It is not known exactly what activates the disease, however three things are required for a person to develop CD:

  • A genetic disposition: being born with the necessary genes. The Human Leukocyte Antigen (HLA) genes specifically linked to celiac disease are DR3, DQ2 and DQ8.
  • A trigger: some environmental, emotional or physical event in one’s life. While triggering factors are not fully understood, possibilities include, but are not limited to adding solids to a baby’s diet, going through puberty, enduring a surgery or pregnancy, experiencing a stressful situation, catching a virus, increasing WBRO products in the diet, or developing a bacterial infection to which the immune system responds inappropriately.
  • A diet: containing WBRO, or any of their derivatives.

 

Summary

Celiac disease is life-long and currently incurable. The only known treatment at this time is strict adherence to a gluten-free lifestyle, free of WBRO.

 

Selected Bibliography

  • Trier, JS, Celiac Sprue, New England Journal of Medicine, 325:1709-1719, 1991.
  • Marsh, MN, Gluten, Major Histocompatibility Complex and the Small Intestine, Gastroenterology, 102:330-354, 1992.
  • Marsh, MN, ed Celiac Disease Methods and Protocols, 2000.
  • Maki, M and Collin, P, Coeliac Disease, Lancet 349:1755-1759, 1997.
  • Sturgess, RP et al, Cereal Chemistry, Molecular Biology and Toxicity in Coeliac Disease, Gut 32:1055-1060, 1991.
  • Sturgess, RP et al, Wheat Peptide Challenge in Coeliac Disease, Lancet, 343:759-761, 1994.
 

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Celiac Disease

 


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