Celiac disease (gluten enteropathy)

Celiac disease (nontropical spruce, gluten enteropathy, celiac disease) is an immunologically mediated disease of the gastrointestinal tract in genetically predisposed individuals, manifested by intolerance to gluten, inflammation of the mucosa and malabsorption. Symptoms of celiac disease usually include diarrhea and feelings of abdominal discomfort. The diagnosis is established by a biopsy of the small intestine demonstrating some nonspecific changes, consisting in pathological atrophy of the villi, with the improvement occurring when a strict gluten-free diet is prescribed.

Synonyms for the term "gluten or gluten-sensitive enteropathy" are celiac disease, celiac disease, celiac disease, idiopathic steatorrhoea, non-tropical sprue. A number of authors consider the term "celiac disease" to be more successful. Many people emphasize that the definition of "gluten enteropathy" is alternative. 

[1], [2], [3], [4]

What causes celiac disease?

Celiac disease is a hereditary disorder caused by increased sensitivity to the gliadin fraction of gluten, a protein found in wheat; similar proteins are found in rye and barley. Genetically susceptible people are activated gluten-sensitive T cells in the case of the presentation of gluten-derived protein determinants. The inflammatory response leads to a characteristic atrophy of the villi of the small intestinal mucosa.

The prevalence of the disease varies from about 1/150 in southwestern Ireland to 1/5000 in North America. The disease of celiac disease is observed in approximately 10-20% of first-generation relatives. The ratio of women and men is 2: 1. The disease manifests itself most often in childhood, but can develop later.

Symptoms of celiac disease

Specific symptoms of celiac disease are absent. In some patients, the disease is asymptomatic or only with symptoms of food deficiency. In others, there may be marked signs from the digestive tract.

In infancy and childhood, celiac disease can occur after the addition of cereals in food. The child has a developmental disorder, apathy, anorexia, pallor, general hypotension, bloating and muscle hypotrophy. The chair is usually soft, plentiful, the color of clay with an unpleasant odor. Older children usually have anemia and dysplasia.

In adults, the most typical symptoms of celiac disease are fatigue, weakness and anorexia. Moderate and unstable diarrhea is sometimes the main symptom. Steatorea manifests itself from moderate to significant (7-50 g fat / day). Some patients experience weight loss, but weight loss is less than normal. Typically, these patients have anemia, glossitis, angular stomatitis and aphthous ulcers. Typical manifestations of deficiency of vitamin D and calcium (eg, osteomalacia, osteogenesis, osteoporosis). Both men and women may have a decrease in fertility.

Approximately 10% have herpetiform dermatitis, a pronounced papular-vesicular rash with itching, which symmetrically captures the extensor surfaces of the elbow and knee joints, buttocks, shoulders and scalp. The appearance of a rash can be caused by eating high gluten content. The development of celiac disease is also associated with diabetes mellitus, autoimmune thyroid diseases and Down's syndrome.

Diagnosis of celiac disease

The diagnosis is suspected in clinical and laboratory changes indicating malabsorption. Family history is an important element in diagnosis. Celiac disease is expected in patients with iron deficiency without obvious gastrointestinal bleeding.

Confirmation of the diagnosis requires a small bowel biopsy from the descending branch of the duodenum. Morphological data include a lack or contraction of villi (villous atrophy), an increase in intraepithelial cells and hyperplasia of crypts. However, such data can also be observed in tropical sprue, severe flow of excess intestinal bacterial growth, eosinophilic enteritis, intolerance of lactose and in lymphoma.

Since the biopsy does not reveal any specific changes, serological markers can help in the diagnosis. The determination of a combination of antigliadine (AGAT) and anti-endomysial antibodies (AEAT - an antibody against the intestinal protein of connective tissue) has a positive and negative predictive value of almost 100%. These markers can also be used for screening populations with a high prevalence of celiac sprue, including the first generation of relatives of diseased patients and patients with diseases that are often associated with celiac sprue. In the case of any positive test, the patient must perform a diagnostic biopsy of the small intestine. If both tests are negative, celiac disease is unlikely. The titer of these antibodies decreases with a gluten-free diet and therefore these tests are effective in monitoring diet compliance.

Often there are other laboratory changes that need to be investigated. They include anemia (iron-deficiency anemia in children and folate-deficient anemia in adults); a decrease in the level of albumin, calcium, potassium and sodium; increase in alkaline phosphatase and prothrombin time.

Malabsorption tests are not specific for celiac disease. But if the studies are performed, the results indicate steatorrhea up to 10-40 g / day, a violation of the test for D-xylose and (with severe ileal damage) Shilling test.

[5], [6], [7], [8]

Treatment of celiac disease

Treatment of celiac disease includes a gluten-free diet (avoid foods containing wheat, rye or barley). Gluten is widely used in food products (eg, in ready-made soups, sauces, ice cream, hot dogs), therefore, in order to avoid the intake of gluten, patients need a detailed description of the food composition ,. The patient needs consultations with a dietitian and the inclusion of patients with celiac disease in the support group. The result after the appointment of a gluten-free diet occurs quickly and the resolution of symptoms occurs within 1-2 weeks. The intake of even small amounts of food containing gluten can lead to an exacerbation or cause a relapse.

A biopsy of the small intestine should be repeated 3-4 months after a gluten-free diet. If changes persist, other causes of villous atrophy (eg, lymphoma) should be considered. The disappearance of celiac disease symptoms and improvement in the morphology of the small intestine are accompanied by a decrease in the titers AGAT and AEAT.

Depending on the deficit, you should prescribe vitamins, minerals and preparations that increase the hemoglobin content. In mild cases, treatment does not require the use of additional drugs, but at the same time, severe manifestations of the disease require full-fledged therapy. In adults, additional treatment includes iron sulfate 300 mg orally one to three times a day, folate 5-10 mg orally once a day, calcium supplements and any standard multivitamin complex. Sometimes, in the severe course of the disease in children and its primary detection (rarely adults), it is necessary to exclude food and parenteral nutrition.

If there is no effect from a gluten-free diet, one should think about the erroneous diagnosis or development of the refractory stage of the celiac disease course. In the latter case, the administration of glucocorticoids may be effective.

What is the prognosis of celiac disease?

Celiac disease ends lethal in 10-30% in case of non-compliance with the diet. If the diet is observed, the mortality rate is less than 1%, mainly in adults, who initially had a serious course of the disease. Complications of celiac disease include refractory course of sprue, collagen sprue and development of intestinal lymphomas. Intestinal lymphomas occur in 6-8% of patients with celiac disease, usually in patients older than 50 years. The risk of malignant growth of other parts of the digestive tract (eg, carcinoma of the esophagus or oropharynx, small intestinal adenocarcinoma) increases. Adherence to a gluten-free diet can significantly reduce the risk of malignancy.