Enteropathy is a pathological condition caused by the absence, deficiency or impairment of intestinal enzymes that provide digestive processes.
Characterized by celiac disease, or gluten enteropathy (non-tropical sprue), gluten intolerance due to a congenital deficiency of its splitting enzymes in the mucous membrane of the small intestine. The causes and mechanisms of development are not fully established.
The disease is manifested by atrophy of the enteric villi, which are restored when gluten is excluded from the diet. Hereditary gluten intolerance is found in the general population in 0.03% of individuals, the disease is often familial in nature. Abnormal sensitivity to gluten causes damage to the intestinal mucosa. Genetic factors are of some importance. In 80% of patients, histocompatibility antigen HLA-B8, associated with the disease, is detected.
Antigenic disorders are inherited by recessive type. The mechanism of development of gluten enteropathy plays a significant role in immune mechanisms. Presumably gluten intolerance is a non-specific toxic reaction that occurs when intestinal absorption of incompletely digested gluten products.
Gluten is a group of proteins contained in wheat, rye, barley, but it is not found in oats, rice, corn. Gluten includes L-gliadin, which is a toxic substance. Lymphokines and antibodies produced by the interaction of gluten with enterocyte receptors and lymphocytes damage the enterocytes of the villi, resulting in (mucous membrane atrophies, infiltrates immunocompetent cells. Atrophy causes impaired absorption of food substances.
Most often absorptive cells of the mucous membrane of the small intestine are affected.
Clinical options: typical form; latent form; atypical severe form with hemorrhagic syndrome, osteomalacia (softening of the bones), myopathy, polyarthralgia (pain in the joints), endocrine disorders, severe retardation of physical development (form resistant to treatment); mild form with selective lack of digestion. Phases of the disease: decompensation, subcompensation (against the background of the gluten-free diet), subcompensation (without the use of diet), compensation.
The following complications may develop:
- pathological fractures,
- adrenal insufficiency,
- infectious diseases.
The main clinical symptoms: diarrhea, steatorrhea, weight loss and bloating, general weakness. Symptoms appear in childhood, then decrease and reappear in 30–60 years.
The following clinical forms exist:
1) typical with the development of the disease at an early age – diarrhea with polyfecalia and steatorrhea, anemia, loss of body weight;
2) erased with a predominance of extraintestinal manifestations – iron deficiency anemia, hemorrhagic syndrome, osteoporosis, polyarthralgia;
3) latent (hidden) with a long subclinical course and first manifested in adults or even in old age.
Celiac disease can occur at any age, it can be suspected based on the following data: impaired development in infancy, growth retardation in childhood, malnutrition in adults.
Symptoms of malabsorption are triggered by pregnancy, infection and surgery. Diarrhea may be absent or intermittent, alternating with constipation. For a long time, patients are worried about drowsiness, bloating, rumbling, abundant foamy with undigested food particles of light brown color with a rancid or fetid odor up to 5 or more times a day.
One of the main symptoms of malabsorption is weight loss. Its severity depends on the severity and extent of damage to the small intestine.
I degree – weight loss does not exceed 5-10 kg from the norm;
Grade II – body mass deficiency of more than 10 kg,
Grade III is accompanied by numerous changes: hypovitaminosis, anemia, trophic disorders, decreased function of the sex glands, weight deficit of more than 20 kg, pronounced water-electrolyte disorders. Due to increased appetite, the loss of body weight is compensated.
Aphthous ulcers are usually formed in celiac disease. It is sometimes possible to detect finger changes in the form of “drumsticks”. The only sign of the disease may be refractory iron deficiency anemia. Elderly patients indicate pain in the muscles and bones, they may have megaloblastic anemia.
Pain syndrome is not characteristic of celiac disease, but nausea and vomiting are possible. With a severe course of the disease, hemorrhagic syndrome develops (petechial hemorrhages on the skin, uterine, nasal, gastrointestinal bleeding); Prothrombin deficiency and violation of intestinal absorption of vitamin K gradually occur. With a deficiency of calcium and magnesium, a violation of sensitivity, muscle cramps, neurological symptoms (muscle weakness, ataxia, peripheral neuropathy, encephalopathy) develop.
Symptoms of the disease during the physical examination of patients – pronounced flatulence, splashing noise with a shaking of the anterior abdominal wall, and can not be found, sometimes only individual signs are found (inflammation of the tongue, aphthae, zadey). But you can find severe manifestations of malabsorption syndrome (exhaustion, edema, ascites). Sometimes with celiac disease, there are also systemic disorders: herpetiform dermatitis (intense itchy skin vesicle papules on the elbows and buttocks), autoimmune diseases (diabetes, thyrotoxicosis, Addison’s disease). These disorders usually disappear if patients switch to a gluten-free diet.
Biopsy of the mucous membrane of the distal duodenal or jejunum is the only reliable way to diagnose, but even if the mucous membrane structure is restored against the background of treatment, the need to exclude microbial contamination of the small intestine, giardiasis remains.
Also, when making a diagnosis of celiac disease, it is necessary to investigate serum immunoglobulins: if there is an IgA deficiency, villus atrophy can be treated with immunoglobulins. Coprological examination is used to determine the severity of malabsorption syndrome. Other causes of villous atrophy include acute viral and bacterial enteritis, giardiasis, excessive bacterial growth, hypogammaglobulinemia, intolerance to cow’s milk and pork, Whipple’s disease, nonsteroidal anti-inflammatory drugs, HIV enteropathy, fasting. Oral carbohydrate tolerance tests help to recognize a disorder in absorption function, but their interpretation can be difficult. Antibodies to gluten are detected by the method of indirect immunofluorescence.
X-ray examination: enlarged loops of the small intestine, the replacement of the normal cirrus pattern of the mucous membrane with a cellular one, the complete disappearance of the folds of the mucous membrane; defeat mainly of the upper small intestine in the absence of changes in the ileum. Bone radiographs show diffuse demineralization with a genetically reduced bone density, and compression fractures are possible.
Possible complications of celiac disease are malignancy (T-cell lymphoma of the small intestine develops in less than 5% of patients, enteric adenocarcine). Suspected malignancy in patients with celiac disease is possible in the presence of recurrence of symptoms, despite adherence to the gluten-free diet, the appearance of abdominal pain and weight loss. Ulceration in the small intestine leads to abdominal pain, the development of anemia and excessive bacterial growth.
The differential diagnosis of celiac enteropathy is carried out with Whipple’s disease, Crohn’s disease, chronic enteritis, diffuse small intestinal lymphoma, viral gastroenteritis, HIV infection.
In the treatment, firstly, it is necessary to completely abandon the ingestion of foods containing gluten. This product includes: rye and wheat bread, cereals and pastries, sausages, canned meat, mayonnaise, ice cream, noodles, chocolate, beer (they may contain a small amount of cereals).
You can eat rice, corn, soybeans, milk, eggs, fish, potatoes, vegetables, fruits, berries, nuts. The inclusion in the diet of meat, butter and vegetable oil, margarine, coffee, cocoa, tea depends on the individual tolerance.
Such a diet reduces the risk of malignancy. The gluten-free diet gives a good therapeutic effect in 85% of patients, but histological restoration of the mucous membrane occurs only after 3–6 months;
It is necessary to undergo annual clinical examination. The lack of noticeable improvement on the background of the gluten-free diet in the first 3 months is the result of non-compliance with the diet, the presence of associated diseases, giardiasis, parasitic diseases, Addison’s disease, small bowel lymphoma, ulcerative inflammation of the jejunum. If there is a weak response to agglomerase therapy and if lactase deficiency is suspected, eliminate dairy products. Perform fluoroscopy of the small intestine with barium to rule out Crohn’s disease, lymphoma, diverticulosis, or adenocarcinoma.
Some patients taking even a small amount of gluten with food leads to cholera-diarrhea, significant dehydration, even shock. You must constantly follow a diet, because hypersensitivity to gluten persists throughout life.