Definition and Overview

Celiac disease is a serious disorder caused by an abnormal immune reaction to gluten, a naturally occurring protein found in wheat and certain grains. When a person with celiac disease consumes gluten-containing food, his or her immune system will attack the small intestine, resulting in damage to the intestinal villi. Villi (singular villus) are finger-like projections in the small intestine that absorb nutrients from foods a person eats. As damage to the villi prevents them from absorbing nutrients, the patient suffers from malnutrition, which could lead to unintended weight loss, loss of muscle mass, anaemia (due to iron deficiency), electrolyte imbalances, serious infections, and even heart failure.

Celiac disease is often confused with wheat allergy but the two conditions are different. The latter is a type of food allergy that is triggered by proteins found in wheat, which include gluten. But unlike celiac disease, it does not damage the small intestine.

Causes of Condition

Just like other autoimmune disorders where the immune system mistakenly attacks healthy cells, the exact cause of celiac disease is unknown. Experts believe that it is the result of a combination of environmental factors, gut bacteria, and gastrointestinal infections.

The condition can develop at any age after a person consumes gluten-containing food or medicine. However, there are cases where it becomes active for the first time or is triggered following a viral infection, certain surgical procedures, childbirth, and severe emotional stress.

The condition is more common in people who have:

  • Addison’s disease

  • Microscopic colitis

  • People with first-degree relatives with celiac disease

  • Rheumatoid arthritis

  • Turner or Down Syndrome

  • Type 1 diabetes

Key Symptoms

Celiac disease impacts almost all body systems because it prevents the body from getting enough nutrients no matter how much food a person eats. These symptoms include:

  • Dental and skin conditions - Eczema, enamel loss, itchy skin rash, hair loss, and mouth ulcers

  • Gastrointestinal problems - Chronic diarrhoea, swollen belly, vomiting, poor appetite, abdominal pain, acid reflux, bloating, lactose intolerance, foul-smelling stool, and unexplained liver problems

  • Musculoskeletal conditions - Muscle wasting, bone or joint pain, arthritis, muscle pain, peripheral neuropathy, and osteoporosis

  • Problems in the reproductive system - Delayed puberty, infertility, miscarriage, and menstrual irregularities

  • General conditions - Anaemia, chronic fatigue syndrome, failure to thrive, malnutrition, and vitamin deficiency

When left untreated, celiac disease can also cause central and peripheral nervous system disorders, gastrointestinal cancers, organ malfunction, and neurological problems.

Who to See and Types of Treatments Available

Celiac disease can be diagnosed with a special blood test that checks for immunoglobulin G (IgG) and immunoglobulin A (IgA) - two types of antibodies that attack the enzyme responsible for gluten breakdown (tissue transglutaminase or tTG). The measurement of the IgA form of tTG antibody through a blood test is specifically useful because it is produced in the small intestine where irritation and inflammation occur. To ensure the accuracy of the test, the patient must be currently consuming gluten-containing food as the antibodies will only be produced if the body is exposed to gluten. This blood test is often combined with endoscopic biopsy in which a gastroenterologist takes a small sample tissue from the small intestine. The sample is then studied under a microscope to find any damage consistent with celiac disease.

Celiac disease treatment focuses on relieving the patient’s symptoms and preventing more complications from occurring. Lifelong adherence to a strict gluten-free diet is advised to prevent further intestinal damage. Patients are also provided with:

  • Vitamins and mineral supplements, which can be taken orally or injected depending on the severity of the patient’s condition

  • Steroids, to control the inflammation of the small intestine

  • Skin medications, for patients who have developed skin conditions

If patients have followed a strict gluten-free diet for up to 12 months but still suffer from the symptoms mentioned above, they are diagnosed with refractory celiac disease (RCD). This requires specialist treatment and can be managed with:

  • Elemental diet - A diet consists of nutrients that do not require digestion such as minerals, vitamins, amino acids, and carbohydrates.

  • Total parenteral nutrition - Feeding the patient with nutritional formulae containing nutrients, lipids, minerals, and vitamins intravenously, bypassing the usual process of eating and digestion.

  • Steroid therapy

  • Immunosuppressive drugs

  • Chemotherapy with or without stem cell transplantation

Patients with RCD require long-term medical care. They are advised to visit their doctor on a regular basis and undergo tests periodically to monitor their progress and ensure that their condition is improving. They must also work closely with a dietitian with experience in managing celiac disease patients.

Prognosis

The long-term outlook for patients can vary widely because not all celiac disease sufferers present with symptoms. In general, however, patients respond well to treatment and after months or years of strictly following a gluten-free diet, the damage to their intestine is resolved and no further damage occurs.

References:

  • Feldman M, et al. Celiac disease. In: Sleisenger and Fordtran’s Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 10th ed. Philadelphia, Pa.: Saunders Elsevier; 2016. http://www.clinicalkey.com.

  • Agency for Healthcare Research and Quality (2004). Celiac Disease (AHRQ Evidence Report/Technology Assessment No. 104). Rockville, MD: Agency for Healthcare Research and Quality

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