Celiac Sprue

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Celiac Diseaseand tropical sprue
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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.

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• 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.

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Signs and Symptoms

• Uncommon
• 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
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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)

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

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Diagnosis of Celiac Disease

• Clinical Findings
• Small Intestines Mucosal Biopsy
• Gluten Re-challenge
• Serologic testing

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Diagnosis Small Bowel Endoscopy
Normal
Celiac
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Histologic Findings of Celiac
Normal Jejunum
Celiac
Virginia Commonwealth Univ, Richmond, Virginia
Celiac Disease (Gluten-Induced Enteropathy)
65000-45800-F2923
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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
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Other Causes of Villous Atrophy

• Bacterial Overgrowth
• Crohns disease
• Cows milk protein intolerance (children)
• Eosinophilic gastroenteritis
• Giardiasis
• Lymphoma
• Post gastroenteritis
• Tropical sprue
• Zollinger Ellison syndrome

American Gastroenterological Association,
Ciclitra, PJ, Gastroenterology 2001 120 1526.
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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 36743.
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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

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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.

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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

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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

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Patients unresponsive to gluten-free diet

• .-Poor dietary compliance
• -Coexistent irritable bowel syndrome
• -Microscopic colitis
• -Lactase deficiency
• -Small intestinal bacterial overgrowth
  Lymphoma
• -Refractory sprue

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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.

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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.

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Morphology

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

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Symptoms

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

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

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Treatment-

• 3 to 6 months of antibiotics (tetracycline)
  and folic acid supplements.