Celiac DiseaseCeliac Diseaseand tropical sprueand 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.

• 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

• Common– Diarrhea– Fatigue– Borborygmus– Abdominal pain– Weight loss– Abdominal distention– Flatulence

• Uncommon– Osteopenia/ osteoporosis– Abnormal liver function– Vomiting– Iron-deficiency anemia– Neurologic dysfunction– Constipation– Nausea

Up to 38 % Asymptomatic

Celiac Disease: Associated Celiac Disease: Associated DisordersDisorders

• 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 Celiac Disease: Dermatitis HerpetiformisHerpetiformis

• 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

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)

Celiac Disease: MalignanciesCeliac Disease: Malignancies

American Gastroenterological Association, Ciclitra, PJ, Gastroenterology 2001; 120: 1526.

Diagnosis of Celiac DiseaseDiagnosis of Celiac Disease

• Clinical Findings

• Small Intestines Mucosal Biopsy

• Gluten Re-challenge

• Serologic testing

Diagnosis: Small Bowel Diagnosis: Small Bowel EndoscopyEndoscopy

NormalNormal CeliacCeliac

Histologic Findings of Celiac Histologic Findings of Celiac

Normal Jejunum

Virginia Commonwealth Univ, Richmond, Virginia Celiac Disease (Gluten-Induced Enteropathy) 65000-45800-F2923

Celiac

Histologic Findings of CeliacHistologic 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 AtrophyOther 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 TestingDiagnosis 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 DiseaseManagement 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 DiseaseManagement 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

• 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

Monitoring Adherence by Serologic Testing

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.