Celiac Disease

-Celiac disease (CD) is a chronic inflammatory, immunologic disease of the small intestine triggered by gluten and related proteins that are components of wheat, barley, and rye grain products.

-The hallmark of celiac disease is an abnormal small-intestinal biopsy.

-Celiac disease is associated with dermatitis herpetiformis, diabetes mellitus type 1, IgA deficiency, Down’s syndrome, and Turner’s syndrome.

-IgA deficiency is 10 to 15 times more common among patients with CD than in the general population.

Symptoms and Signs:  

Gastrointestinal: Chronic diarrhea, steatorrhea, abdominal distention, irritability, anorexia, vomiting, and poor weight gain are typical.

Non-gastrointestinal:  iron-deficiency anemia, decreased bone mineral density, osteoporosis, infertility, elevated liver function enzymes, arthritis, or epilepsy with cerebral calcifications, neurological symptoms such as ataxia, neuropathy, migraine or rash suggestive of dermatitis herpetiformis. Celica Crisis can produce dehydration, hypotension, and hypokalemia.

Dermatitis herpetiformis is a form of gluten sensitivity characterized by a pruritic, papulovesicular rash that affects up to 20% of CD patients.  

Diagnosis:

The most sensitive and specific tests are antitissue transglutaminase (anti-tTG) or antiendomysial IgA antibodies. However, the gold standard of diagnosis is still demonstration of villous atrophy, flattening of the mucosa, increased intraepithelial lymphocytes on duodenal biopsy and a clinical response to a gluten-free diet.

-The preferred single, initial testing: A serologic test for IgA antitissue transglutaminase (tTG) antibodies.

-Genetic testing for HLA-DQ2 or DQ8 has strong negative predictive value and can help to rule out CD when the diagnosis is uncertain.

-Patients should be tested for deficiency of vitamins (A, D, E, B12), copper, zinc, carotene,folic acid, ferritin, and iron.

Treatment:

Diet: The mainstay of treatment for CD is lifetime adherence to a gluten-free diet. Avoid wheat, rye or barley.

Corticosteroids: Corticosteroids are indicated for celiac crisis with profound anorexia, edema, abdominal distention, and hypokalemia.

Nutrients: Iron, Folate, Calcium, Vitamin D

Vaccines: Pneumococcal vaccine to those with evidence of hyposplenism.

Prognosis: The most important complication of celiac disease is the development of cancer: T cell Lymphomas, gastrointestinal neoplasms such as small intestinal adenocarcinoma

Q: What is the most reliable, initial, noninvasive test to diagnose CD? Tissue transglutaminase (tTG) IgA antibodies

Q: What do you suspect in a patient with typical symptoms of CD who is negative for Immunoglobulin A (IgA) anti-tissue transglutaminase (tTG) antibody? IgA deficiency.

Q: What is the gold standard to establish the diagnosis of CD? Intestinal biopsy.

Q: What are the most important known genetic contributors to the pathogenesis of CD? genes encoding the MHC class II molecules, HLA-DQ2 or HLA-DQ8.

Q: Does Dermatitis herpetiformis respond to gluten withdrawal? Yes

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