What Is Celiac Disease (Celiac Sprue)?

Celiac (Greek koiliakos = abdominal) disease is an inherited malabsorption disorder of the small intestine, in which symptoms like diarrhea and bloating are triggered by ingesting gluten – a common name for proteins, present in wheat, barley, rye and possibly oats. 

Celiac disease may be mild or severe, and may affect children or adults. It is considered as a life long disease, and requires permanent gluten-free diet to be controlled.

Other names for celiac disease: celiac sprue, non-tropical sprue and gluten-sensitive entheropathy.

Autoimmune Mechanism of Celiac Disease

Immune system of a sensitive person recognizes gluten (and especially its part gliadin) as ‘foreign substance’, and attacks it with immune cells and antibodies. These immune cells and antibodies destroy cells in villi (microscopic finger-like projections from small intestinal mucosa) thus greatly reducing its absorption surface. Absorption of fats, vitamins B12 and D, calcium, and iron is mainly affected. Production of lactase, an enzyme that digests milk sugar lactose, may also be reduced, so lactose intoleranceoften develops in celiac disaese.

Celiac disease is not a food allergy (where released histamine causes itch, facial swelling, etc), but an autoimmune disorder where antibodies and immune cells react against body’s own tissues.

Celiac disease is also not the same as gluten intolerance.

Who Gets Celiac Disease?

Celiac disease may affect any predisposed person at any age – although it most often appears in children. Three conditionsare needed for celiac disease to develop:

  1. Genetic predisposition. Only people with a certain DNA change are prone to celiac disease. If one family member has celiac disease, one out of ten other family members will also likely have it.
  2. Triggering event. Emotional stress, surgery, injury, pregnancy, childbirth, or viral infection, may lead to change in permeability of intestinal mucosa permeability, so gluten can enter in deeper mucosal layers where it causes inflammation. Sometimes a triggering event can’t be identified.
  3. Ingesting gluten. Even tinny amount of gluten may cause intense intestinal damage, if regularly ingested.

Symptoms of Celiac Disease

Severity of small intestinal mucosal damage does not correlate with severity of symptoms. Some people, despite prominent intestinal changes, have no symptoms at all.  Symptoms of celiac disease may vary a lot from person to person, and may include (7):

  • Diarrhea (starting few hours to few days after eating gluten-containing food), constipation, bloating and gas, abdominal pain, nausea, heartburn, mouth ulcers
  • Weight loss (not always!)
  • Headache
  • Pale skin, tiredness (due to anemia due to iron deficiency)
  • Easy bruising(due to vit K deficiency)
  • Hair loss - alopecia (due to vit A deficiency)
  • Skin rash – dermatitis herpetiformis (picture)
  • Joint or bone pains
  • Depression

Complications of Celiac Disease:

  • Defective tooth enamel in children
  • Osteoporosis in adults, and growth impairment in children (due to calcium deficiency)
  • Poor muscle co-ordination (ataxia), numbness and tingling in the hands and feet(peripheral neuropathy), problems with vision, epilepsy and dementia 
  • Infertility, recurrent miscarriages
  • Small intestinal adenocarcinoma or lymphoma are rare complications.

Diagnosis of Celiac Disease

Diagnosis of celiac disease is confirmed by:

  1. Symptoms, improving after starting gluten-free diet
  2. Biopsy of duodenal mucosa reveals characteristic destruction of villi – microscopic finger like protrusions of mucosa into intestinal lumen  (1), (Picture 1)

Celiac Disease - Endoscopy and Histology

Picture 1. UPPER ROW: Normal intestinal mucosa: endoscopic (A) and microscopic (B) image.
LOWER ROW: Celiac disease: fissures in the intestinal mucosa (C), lost villous structure (D)

Other tests:

  • Specific igA antibodies in the blood(anti-endomysium (EMA -IgA), and anti-tissue transglutaminase (tTG IgA) antibodies) are usually checked first. Positive result is not a proof of celiac disease, so duodenal biopsy usually follows.
  • Stool IgA anti-gliadin antibody test isn’t reliable.
  • DNA test100% exclude diagnosis of celiac disease, if it’ is negative, but does not confirm celiac disease if it is positive. All patients with celiac disease have specific genes (HLA DQ2 or DQ8) in their DNA. A person with these genes doesn’t necessary have a celiac disease, but it has increased risk of developing it. Absence of specific genes excludes celiac disease and possibility of its development in future. DNA analysis is recommended to all 1st grade relatives of patients with celiac patients. If specific HLA genes are found, serum antibodies may be checked to detect eventual active disease in the early stage, before complications (growth impairment, osteoporosis) develop.
  • Blood workcan show: anemia, deficiency of iron, calcium, vit D, and B12.
  • Increased fats are often found by the stool fat test. 
  • Dual Energy X-ray Absorptiometry (DEXA) scan, to evaluate bone density and find eventual osteoporosis.

Prevention of Celiac Disease

A gluten-free diet is effective in most cases; symptoms usually disappear completely within 3-6 months in children and within 2 years in adults (2). Some patients don’t improve despite gluten-free diet, most often because they don’t stick with it strictly. The diet should be life-long, otherwise the disease may return. Infants exposed to gluten before 3rd month of age are at greater risk of developing celiac disease later in life, so exclusive breastfeeding until the 6th month is recommended (3). Local celiac support groups or online forums may be a good source of advice about the diet and recipes (4). A skilled dietitian should be visited for the most reliable information though.

Diet for Celiac Disease

Foodstuffs, Containing Gluten, NOT SAFE to Eat in Celiac Disease:

  • WHEAT(also bulgur, couscous, dinkle, durum, einkorn, emmer, farina, fu, graham flour, granary flour, grits, groats, hemp, kamut, matzo, mir, seitan, semolina, spelt, triticale, udon);
  • BARLEY (malt, flavorings, beer, ale, gin, whisky);
  • RYE
  • OATS (most of commercially available oats are ‘contaminated’ with wheat, barley, or rye grains).

Gluten may be “hidden” in bran, food additives like edible starch, thickeners, preservatives, stabilizers used in frozen foods, licorice, syrups, chips, sauces, salad dressings, candies, instant foods, hot dogs, and other processed foods. Gluten is even in some medicines, cosmetics, and stamps (!). Labels usually tell if a product contains gluten or not.

Sharing food-preparing surfaces, utensils, dishes and other kitchenware with others may contaminate gluten-free food.

Persons with celiac disease may be lactose intolerant or sensitive to molds in mushrooms, mold-based cheeses, air-born molds (5).

Grains, SAFE TO EAT in Celiac Disease:

  • Amaranth
  • Buckwheat
  • Corn
  • Millet
  • Montina
  • Quinoa
  • Rice
  • Sorghum
  • Teff

Seeds, nuts, legumes and tubers (potato) are also safe (6). Gluten-free bread, pasta and other foods are available in many food stores on separate shelves.

Therapy of Celiac Disease

No therapy for celiac disease exists at the time. Vitamins and mineral replacement may be needed. Drinking enough fluid to prevent dehydration and providing enough calories is needed for maintaining proper body weight.

Refractory Celiac Disease

In some persons with celiac disease, the intestine is so damaged, that it can’t recover, despite the appropriate diet. This is called refractory celiac disease; corticosteroid therapy may help, and sometimes intravenous feeding is necessary (6).

Complications of Celiac Disease

Complications of celiac disease may include:

  • Iron deficiency anemia
  • Vitamin B12 deficiency
  • Osteoporosis in adults, and growth impairment in children
  • Neurological complications: peripheral neuropathy, myelopathy, optic myopathy, epilepsy and dementia. 
  • Small intestinal adenocarcinoma or lymphoma are rare complications.

Helpful Links:

References:

  1. Diagnostic criteria for celiac disease   (edrv.endojournals.org)
  2. Prognosis of celiac disease  (digestive.niddk.nih.gov)
  3. Breast feeding and celiac disease  (jama.ama-assn.org)
  4. Gluten forum  (forums.glutenfree.com)
  5. Sensitivity to molds in celiac disaese  (csaceliacs.org)
  6. Safe foods in celiac disese, refractory celiac disease  (celiac.org)
  7. Symptoms of celiac disease  (health.nsw.gov.au)
  8. Blood test: anti-endomysium and anti-tissue transglutaminase antibodies  (celiacdisease.net)

Further Reading :
  • Gluten Intolerance, Wheat Intolerance, Wheat Allergy, Celiac Disease