Transcript Celiac Sprue

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.
• 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
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Diarrhea
Fatigue
Borborygmus
Abdominal pain
Weight loss
Abdominal distention
Flatulence
Up to 38 % Asymptomatic
• Uncommon
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Osteopenia/ osteoporosis
Abnormal liver function
Vomiting
Iron-deficiency anemia
Neurologic dysfunction
Constipation
Nausea
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
Celiac Disease: Malignancies
Malignancy
All cancers
Overall
Relative Risk
2 to 3
Enteropathy associated T-cell
lymphomas
30 to 40 (w/o
gluten free
diet)
Small intestinal
adenocarcinoma
83
Mouth, pharynx,
esophagus cancer
American Gastroenterological Association, Ciclitra, PJ, Gastroenterology 2001; 120: 1526.
23 (w/o gluten
free diet)
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
Virginia Commonwealth Univ, Richmond, Virginia Celiac Disease (Gluten-Induced Enteropathy) 65000-45800-F2923
Celiac
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
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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
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Bouillon Cubes
Canned soups
Cheese spreads
Chips and dips mixes
Hot chocolate mixes
or cocoa
• Ice cream
• Meat sauces
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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
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-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.