celiac disease

celiac disease

Celiac disease is a disease caused by increased susceptibility to gluten, characterized by atrophy of the mucous membrane of the upper small intestine. Frequency. The prevalence of the disease in different regions ranges from 1:300 to 1:2000. Prevailing age: two peaks of incidence – at 1 year and at 30-60 years. Women are diagnosed 10-15 years earlier (early manifestations in the form of amenorrhea and anemia in pregnancy). Etiology and pathogenesis are not fully understood.

• • Abnormal gluten sensitivity causes damage to the intestinal mucosa
  • The significance of genetic factors is proved by changes in the small intestine, detected by biopsy in 10-15% of close relatives.
  • Familial cases described (212750, 6p, GSE gene, CD, p or polygenic)
  • In 60-90% of patients, leukocyte antigen B8 (HLA-B8) and HLA-DW3 are detected (in the general population, these antigens are detected in 20-30% of individuals)
  • Additional B-lymphocytic Ag is detected in 70-80% of patients (in the general population – in 15%)
  • The pathogenesis common with celiac disease is another gluten-sensitive disease – familial dermatitis herpetiformis. Both diseases are associated with the same leukocyte antigens.

Pathomorphology

• • Shortening of the villi of the small intestine
  • Hyperplasia and deepening of intestinal crypts
  • Infiltration by plasma cells and lymphocytes of the own layer of the mucous membrane.

Clinical picture

• • Diarrhea – profuse, frothy, smelly stools (in 100% of cases)
  • Steatorrhea
  • Bloating (combined with thin limbs [due to muscle atrophy] – appearance of a spider)
  • Malabsorption syndrome – IDA, edema (due to hypoproteinemia), weight loss with good appetite, bone pain, paresthesia, weakness, high fatigue
  • Infertility, recurrent aphthous stomatitis, dermatitis herpetiformis as primary manifestations
  • Young children often have abdominal pain, nausea, vomiting
  • Babies have a developmental delay.

Research methods

• • Peripheral blood test
  • An increase in the content of antigluten IgA and IgG in 90% of patients, a decrease in IgM in 33% of patients
  • Decrease in the content of C3 and C4 complement components in patients in the absence of treatment and an increase in these fractions when prescribing a gluten-free diet
  • Decrease in calcium, potassium, sodium, iron, neutral fats, cholesterol, vitamin A, vitamin B12, vitamin C, folic acid
  • Decreased total protein content
  • Increasing PV.
  • Examination of feces for fats: they reveal a violation of the absorption of fats, the residual amount of fat in the feces is more than 7% (normal – no more than 3-5%).
  • Gliadin loading test – a rapid increase in blood levels of glutamine after the introduction of gliadin at a dose of 350 mg / kg orally.
  • Endoscopy with biopsy. The diagnosis is considered reliable only after a triple biopsy.
  • The first biopsy (at the height of the disease) – shortening of the villi, deepening and expansion of the crypts, the appearance of a cubic epithelium, infiltration of the mucosal layer by inflammatory cells
  • The second biopsy (against the background of a gluten-free diet for 6 months) – restoration of the normal structure of the epithelium
  • The third biopsy (following the appointment of gluten 10 g / day for 6 weeks) – the reappearance of mucosal changes.

Differential Diagnosis

• • short bowel syndrome
  • pancreatic insufficiency
  • Crohn’s disease
  • Whipple disease
  • Sprue tropical
  • Intestinal lymphoma
  • HIV infection and AIDS
  • Acute gastroenteritis
  • Giardiasis
  • Eosinophilic gastroenteritis.

Treatment: Management Tactics

• • Diet number 4a 
  • Exclude the use of gluten (found in wheat, rye, barley, oats), sometimes lactose (probably the development of secondary lactase deficiency)
  • You can take animal products, as well as corn, soy and rice flour, potatoes, vegetables, fruits, berries.
  • In severe cases, corrective products are used
  • Calcium gluconate 5-10 g/day
  • Vitamin D (ergocalciferol) 0.01-1 mg/day (up to 2.5 mg/day with severe malabsorption)
  • Ferrous ferrous sulfate 300 mg/day
  • Folic acid 5-10 mg/day
  • Multivitamin products
  • With a change in PV – vitamin K 10 mg / day / m.
  • Infusion therapy – protein products, fat emulsions, 10-20% glucose solution, correction of water and electrolyte balance and acid-base balance.
  • With an extremely serious condition of patients (more often babies), the transition to parenteral nutrition.
  • With refractory celiac disease – glucocorticoids, for example, prednisolone 10-20 mg 2 r / day orally.

observation. Repeat endoscopy 6 months after the introduction of a gluten-free diet and after 6 weeks of dosed gluten.

Complications

• • Malignant tumors develop in 10-15% of patients (50% of these tumors are lymphoma of the small intestine, esophageal carcinoma often develops)
  • Refractory celiac disease – resistant to a gluten-free diet
  • Collagen celiac disease – the formation of a thick layer of collagen in its own mucosal layer
  • Chronic ulcerative enteritis (formation of multiple ulcers, intestinal bleeding, strictures, perforations, obstructions, peritonitis) – mortality reaches 7%
  • Secondary osteoporosis due to decreased absorption of vitamin D and calcium
  • Exicosis and electrolyte imbalance. The course and prognosis are favorable with timely diagnosis and strict adherence to the diet. Improvement begins within a few days after eliminating gluten from food. All symptoms traditionally disappear after 4-6 weeks. The main underlying cause of death is the development of lymphoreticular pathology (especially intestinal lymphoma). So far, no clear correlation has been found between the incidence of malignant tumors of the gastrointestinal tract and dietary intake. Concomitant pathology
  • Secondary lactase deficiency
  • Herpetiform dermatitis.

Age features

• • Children. Symptoms of celiac disease appear in infancy when foods containing gluten are introduced into the child’s diet.
  • Elderly. Celiac disease often recurs
  • In adolescence, the disappearance of hypersensitivity to gluten is likely.

Prevention. Avoid eating foods containing gluten.

Synonyms

• Sprue non-tropical
• celiac disease
• Sprue european
• Adult celiac disease
• Steatorrhea idiopathic
• gluten deficiency disease
• Celiac disease gluten-sensitive See also Crohn’s disease, Whipple’s disease, HIV infection and AIDS, Giardiasis, Dermatitis herpetiformis, Intestinal lymphoma, Short bowel syndrome ICD K90.0 Celiac disease MIM. 212750 Celiac disease