Celiac Disease PDF

 Celiac disease, called non tropical sprue, celiac sprue or gluten sensitive entheropathy. CD is a genetically determined, immune- mediated disorder in which gluten damages the lining of the small intestine. 60% of cases undiagnosed or misdiagnosed with IBS, lactose intolerance, IB...

 Celiac disease, called non tropical sprue, celiac sprue or gluten sensitive entheropathy. CD is a genetically determined, immune- mediated disorder in which gluten damages the lining of the small intestine. 60% of cases undiagnosed or misdiagnosed with IBS, lactose intolerance, IBD, stress…  Gluten is the general name for specific proteins called prolamins in wheat, barley and rye. Diagnosis often delayed for a mean of 11 years Removing gluten from the diet results in clinical and histological improvement, while reintroducing gluten causes a relapse in villi damage.  Life-threatening complications and higher risk of mortality. Higher risk of enteropathy-T-cell lymphoma or carcinoma of the oropharynx, esophagus and small intestine. At risk first degree relatives and patients with DM should be screened for CD.  Age at presentation: 6 months- > 85 y. Most cases diagnosed in adulthood (20% after 60 y). Infants: – Present between 4-24 m. when cereals are introduced to the diet. – Impaired growth, diarrhea, abdominal distention. – Decrease in W gain → W loss – If untreated → pubertal delay, short stature, IDA, rickets.  Adults: – Symptoms of malabsorption: diarrhea, steatorrhea, anemia, vitamin deficiency, weight loss (but 50% do not have clinically significant diarrhea). – In case of partial villous atrophy: No malabsorption Fatigue, depression, arthralgia, milk intolerance, osteomalacia, osteoporosis, IDA (hypochromic microcytic: most common presentation).  Delay between patient presentation and diagnosis of CD (misdiagnosis with IBS). Presentation during pregnancy or in postpartum period (+++ if severe anemia develops).  Dermatitis herpetiformis: – Occasional – Blistering, itchy skin rash, may be the only presenting symptom, occurring in about 5% of patients with CD between 15–40 years old. – Typically, the rash appears bilaterally on elbows, knees, buttocks and other pressure points on the body. – Patients with DH may not present with gastrointestinal symptoms; however, 70-80% of patients have coexisting damage in the intestine (biopsy). – Treated by dapsone and GF diet Improvement may require from six months to over two years before medication can be discontinued.  Laboratory test abnormalities: def. in iron, folate, Ca, vit A, D, E, K, B12 (non specific abnormalities and need further testing). Blood count reveals anemia Blood smear reveals target cells and Howell-Jolly bodies (hyposplenism). The best non invasive test: serologic markers – Ig A antiendomysial antibody (anti-self) – Ig A and Ig G antigliadin antibodies (anti-gluten). – Tissue transglutaminase antibody (anti self) (most important single indicator). Prior to testing, the patient should not reduce his intake of gluten. Howell-Jolly bodies  The gold standard for final confirmation of the diagnosis is still the intestinal mucosal biopsy. 3 samples taken from the second or third portions of the duodenum. Characteristics: Intraepithelial lymphocytes Crypt hyperplasia Increased plasma cells and lymphocytes in lamina propria Sometimes, severe villous atrophy.  Cornerstone of treatment: gluten-free diet. – No offending grains or any food containing their derivatives. – Wheat: gliadin. – Rye: secalin. – Barley: hordein – Attention to hidden sources of gluten or hydrolyzed plant protein (HPP) that does not list the source of the protein (corn, soy or wheat) may contain gluten.  Note: products listed as “wheat free,” are not necessarily “gluten-free.” – They may contain spelt, kamut or barley (toxic). Barley is commonly used as a flavoring agent in malt form and can be easily hidden in foods. Although it may be listed as “barley malt, barley malt extract or barley malt flavoring,” it may only be listed as flavoring or natural flavoring, making it difficult to decipher the origin.  Controversy about the oat prolamin (avenin) Considered safe but difficult to obtain oats not contaminated with gluten-containing grains → should be avoided in newly diagnosed CD until remission is achieved on a GF diet. → Introduce up to 2 oz per day pure uncontaminated oats.  Safe food include: – Plain meats, fish and poultry. – Milk – Fresh fruits and vegetables, plain frozen, and juices. – Butter, vegetable oils, gravy, creams, white sauces with allowed flours – Gelatin, homemade custard, meringue, GF cakes… – Grains: rice, corn, soy, millet, buckwheat, corn, rice cereals… – Sugar, jelly, jam, honey Food group OK Questionable AVOID MEAT Fresh, frozen, salted, Luncheon meats, Products with gravies smoked sandwich spreads, meat loaf MILK Milk, buttermilk, plain Milk shakes, flavoured Malted milk yogurt, cheese yogurt BREADS, CEREALS Products made with Oat Wheat, barley, rye corn, tapioca, products quinoa… FRUITS & VEGGIES Any unprocessed French fries Vegetables dipped in batter Desserts Ice cream, sherbet, Chocolate bars… Puddings thickened gelatin desserts with wheat Beverages Coffee, tea, cocoa Chocolate drinks… Beer  Avoid Unless Labeled Gluten-free or Made with Corn, Rice, Soy or Other Gluten-Free Grain: Beer Pastas Breads Processed luncheon meats Cakes and pies Salad dressings Candies Sauces, including soy sauce Cereals Seasoned rice mixes Cookies and crackers Seasoned snack foods, such as potato and tortilla chips Croutons Self-basting poultry French fries Soups and soup bases Gravies Vegetables in sauce Imitation meat or seafood Play-dough Gluten-Free Products in the Lebanese Market  < 50 years: 25 and 38 g/d (women and men respectively) > 50 years: 21 and 30 g/d (women and men respectively). Ways to increase fiber intake: – Adding ground flax seed to cold or hot cereal, yogurt and baked goods. – Switching from white rice to brown rice. – Making soups or main dishes from beans and legumes. Increase in parallel water/fluid intake.  Some GF foods are being reformulated to include healthier ingredients and enriching them with a variety of vitamins and minerals (e.g. brown rice flour, quinoa, flax, buckwheat…)  CD patients should pay attention to cross contamination when sharing utensils of others and when dining out and shopping  Celiac patients may have other intolerances +++ a secondary lactose intolerance (if they are not treated [nausea, bloating, diarrhea]) → lactose restricted diet for one month or more to restore the villi and the action of lactase. – Select lactose-reduced or lactose-free products, such as Lactaid milk. – Use GF lactase enzyme supplements (e.g., Lactaid® caplets or chewables or Lacteeze® drops or tablets) when consuming dairy products. – Choose dairy-free/GF beverages, such as soy, almond, or rice milk.  These drinks should be enriched with calcium, vitamin D and other nutrients. Aged cheese and yogurt with live, active cultures are usually well tolerated. Additional oral calcium and vitamin D supplementation. A baseline DEXA scan in all newly diagnosed patients has been suggested.  In CD, the entire small bowel can be affected. In patients who present with diarrhea, steatorrhea and weight loss→ baseline fat-soluble vitamin status should be evaluated. – Vitamins A and E may be needed – Vitamin K may be prescribed for bleeding, or prolonged prothrombin time  Calcium and vitamin D administration may be necessary to correct osteoporosis or osteomalacia – Measure bone density at the time of diagnosis for possible osteoporosis: – 1000-1500 mg Ca – 400-800 IU vit D – Daily exercise – Avoid tobacco and alcohol – HRT in menopausal women with CD  Low serum concentrations of iron, folate, Ca, vitamin D…. with iron-deficiency anemia being the most common clinical presentation in untreated adults. – → iron, folate, or vitamin B12 supp. depending on its nature – Include GF iron-rich foods (lean red meat, poultry and fish, nuts, seeds, flax seed, legumes, dried fruits, several GF grains (amaranth, bean flours, quinoa, rice bran, soy flour) and blackstrap molasses – Add vitamin C-containing food or beverage, such as oranges, tomatoes,… Vitamin B12 deficiency is less common, as distal small bowel disease is unusual.  Symptoms resolve within two weeks after starting a GF diet. A temporary lactose-reduced or lactose- free diet may be indicated and adequate fluid intake encouraged. Other patients may suffer from constipation when starting a GF diet → increase fiber and fluid intake.  Underlying common genetic predisposition. Some patients with DM may present with typical gastrointestinal symptoms, growth retardation or anemia; however, the majority are asymptomatic for CD. Undiagnosed CD in a patient with DM → poor or erratic glycemic control Recommendation: routine screening for CD during the first years after the diagnosis of Type I DM.  Key nutritional strategies: – Monitor blood glucose frequently. – Keep a food diary to assess the effect of various GF foods on glycemic control. – Use carbohydrate counting for meal planning. – Include a solid protein choice at each meal. – Incorporate more fiber-rich GF foods. – Ensure adequate amounts of calcium and vitamin D in the diet and/or use supplements. – Include more iron-rich GF foods.  In cases of severe malabsorption and/or weight loss, patients may need additional oral supplementation to promote weight gain. There are a variety of over-the counter GF commercial liquid nutritional products: Ensure; Boost, Scandishake… Pay attention to the possibility of gluten presence in medications.  If no response to the diet, most common explanation is non compliance. In case of secondary hyposplenism: – Pneumococcal vaccination – Medical evaluation for any suspected infection Parameter Duration for healing Symptomatic improvement 2 weeks Mucosal healing (→ gradual reintroduction of 3-6 months dairies) IgA endomysial and tissue transglutaminase 6 months antibodies undetectable (IgG antigliadin may still be present after 1 year) Dermatitis herpetiformis (patients can 18 months discontinue medications) Repeat densitometry After 1 year Biopsies Every 5 years  Persistent symptoms of malabsorption despite GF diet for at least 6 months with continued villous atrophy on duodenal biopsy. Antiendomysial and antigliadin should be negative. Mainly elderly At risk of developing or coexisting complications (lymphoma…)/ cryptic enteropathy associated T-cell lymphoma. Tx: corticosteroids, immunosuppressive agents.