Celiac Disease and tropical sprue

Celiac disease Inappropriate immune response to the dietary protein gluten, which is found in rye, wheat, and barley. After absorption in the small intestine these proteins interact with the antigen-presenting cells in the lamina propria causing an inflammatory reaction that targets the mucosa of the small intestine. Manifestations range from no symptoms to overt malabsorption with involvement of multiple organ systems and an increased risk of some malignancies. Not completely understood how gluten sensitivity begins or whether early exposure to gluten proteins increases the risk of sensitivity

Most all patients with celiac disease express (HLA)-DQ2 or HLA-DQ8, which facilitate the immune response against gluten proteins Concordance rates of 70 to 75 % among monozygotic twins and 5 to 22 % among first-degree relatives.

Signs and Symptoms Uncommon Common Osteopenia/ osteoporosis Abnormal liver function Vomiting Iron-deficiency anemia Neurologic dysfunction Constipation Nausea Common Diarrhea Fatigue Borborygmus Abdominal pain Weight loss Abdominal distention Flatulence Up to 38 % Asymptomatic

Celiac Disease: Associated Disorders Dermatitis Herpetiformis Iron deficiency anemia Osteoporosis, Osteomalacia and Vitamin D deficiency Malignancies Type 1 diabetes Other autoimmune endocrine disorders Neuropsychologic Features Others (Downs syndrome, IgA deficiency, rheumatologic disorders)

Celiac Disease: Dermatitis Herpetiformis Symmetric vesicles, crusts and erosions distributed over the extensor areas of the elbows, knees, buttocks, shoulders and scalp, with a tendency to grouping of individual lesions. PRUESSNER, HT. Detecting Celiac Disease in Your Patients. 1998 by the American Academy of Family Physicians University of Texas Medical School at Houston

30 to 40 (w/o gluten free diet) Celiac Disease: Malignancies Malignancy  Overall Relative Risk All cancers 2 to 3 Enteropathy -associated T-cell lymphomas 30 to 40 (w/o gluten free diet) Small intestinal adenocarcinoma 83 Mouth, pharynx, esophagus cancer 23 (w/o gluten free diet) American Gastroenterological Association, Ciclitra, PJ, Gastroenterology 2001; 120: 1526.

Celiac Disease: Other Associated Disorders Aphthous stomatitis- unexplained oral ulcers have been reported as the sole presenting feature Glossitis, angular stomatitis, and cheilosis have also been associated

Diagnosis of Celiac Disease Clinical Findings Small Intestines Mucosal Biopsy Gluten Re-challenge Serologic testing

Diagnosis: Small Bowel Endoscopy Normal Celiac

Histologic Findings of Celiac Normal Jejunum Celiac Virginia Commonwealth Univ, Richmond, Virginia Celiac Disease (Gluten-Induced Enteropathy) 65000-45800-F2923

Histologic Findings of Celiac The lamina propria shows a marked increase in the number of plasma cells and lymphocytes and transepithelial migration of lymphocytes across the surface epithelium (arrow) is common. Virginia Commonwealth Univ, Richmond, Virginia Celiac Disease (Gluten-Induced Enteropathy) 65000-45800-F2923

Other Causes of Villous Atrophy Bacterial Overgrowth Crohn’s disease Cow’s milk protein intolerance (children) Eosinophilic gastroenteritis Giardiasis Lymphoma Post gastroenteritis Tropical sprue Zollinger Ellison syndrome American Gastroenterological Association, Ciclitra, PJ, Gastroenterology 2001; 120: 1526.

Diagnosis of Celiac: Serologic Testing IgA antigliadin antibodies Sensitivity 80 to 90 % Specificity 85 to 95 % IgA endomysial antibodies Sensitivity 85 to 98 % Specificity 97 to 100 % IgA tissue transglutaminase antibodies Sensitivity 90 to 98 % Specificity 95 to 97 % Kelly, CP. Coeliac disease: Non-invasive tests to screen for gluten sensitive enteropathy and to monitor response to dietary therapy. Dublin University, Trinity College, Dublin 1995. Kelly, CP, Feighery, CF, Gallagher, RB, et al. Mucosal and systemic IgA anti-gliadin antibody in celiac disease. Contrasting patterns of response in serum, saliva, and intestinal secretions. Dig Dis Sci 1991; 36:743.

Management of Celiac Disease Gluten avoidance is the mainstay of treatment Prior to the introduction of a strict gluten-free diet, prognosis was very poor

Management of Celiac Disease In general, the following advice can be given to all patients: Foods containing wheat, rye, and barley should be avoided. Soybean, rice, corn, and potatoes are safe. Read labels on prepared foods carefully (many stabilizers or emulsifiers contain gluten) Dairy products may need to be avoided initially- many patients have secondary lactose intolerance.

Foods That May Contain Gluten Bouillon Cubes Canned soups Cheese spreads Chips and dips mixes Hot chocolate mixes or cocoa Ice cream Meat sauces Peanut butter Processed canned meats and poultry Soup mixes Tomato sauces Sausages Yogurt with fruit

Monitoring Adherence by Serologic Testing A pretreatment antibody level should be determined at the time of diagnosis. Serologic testing is of no use if antibody levels are not elevated prior to therapy. Exclusion of gluten from the diet results in a gradual decline in serum IgA antigliadin and IgA tTG levels. A normal baseline value is typically reached within three to six months. If the levels do not fall as anticipated, the patient may be continuing to ingest gluten either intentionally or inadvertently

Patients unresponsive to gluten-free diet . -Poor dietary compliance -Coexistent irritable bowel syndrome -Microscopic colitis -Lactase deficiency -Small intestinal bacterial overgrowth Lymphoma -Refractory sprue

What is the tropical sprue? Tropical sprue: is a malabsorption disease commonly found in the tropical regions, marked with abnormal flattening of the villi and inflammation of the small intestinal mucosa.

Causes No specific causal agent has been clearly associated with tropical sprue, but bacterial overgrowth by enterotoxigenic organisms ( e.g., E.coli and hemophilus ) has been implicated.

Morphology Intestinal changes range from near normal to severe diffuse enteritis. Unlike celiac sprue, injury is seen at all levels of the small intestine.

Symptoms The symptoms of tropical sprue are: - Diarrhea. - Indigestion. - Cramps. - Weight loss and malnutrition. - Fatigue.

Investigations: Low levels of vitamins A, D, E, K, and B12 as well as albumin, calcium, and folate. Excess fat in feces

Treatment:- 3 to 6 months of antibiotics (tetracycline) and folic acid supplements.