What is Esophageal Amyloidosis?
Amyloidosis of the esophagus is more common simultaneous lesions of other parts of the digestive system.
Causes of Amyloid Gullet
The development of amyloidosis is associated with a violation of the protein-synthetic function of the reticulo-endothelial system, the accumulation in the blood plasma of abnormal proteins that serve as autoantigens and cause the formation of autoantibodies. As a result of the interaction of the antigen with the antibody, coarse-dispersed proteins are involved in the formation of amyloid. Deposing in tissues (for example, in the walls of blood vessels, glandular, etc.), amyloid replaces functionally specialized elements of the organ, which leads to the death of this organ.
Pathogenesis during Amyloidosis of the Esophagus
AL-amyloidosis (immunoglobulin light chains derived) – primary amyloidosis caused by the accumulation in the blood plasma of abnormal light chains of immunoglobulins synthesized by malignant B-lymphocytes in myeloma (Rustitsky-Kalera disease)
AA-amyloidosis (acquired) is a secondary amyloidosis caused by hypersecretion of the liver of the acute phase alpha globulin by the liver in response to chronic (usually purulent) inflammation. An example of such inflammations is leprosy, bronchiectasis, tuberculosis, etc. A subspecies of this amyloidosis is ASC-amyloidosis (systemic cardiovascular), which develops in people over 90 years old. The causes of pathogenesis are unknown.
AF-amyloidosis (Mediterranean intermittent fever) is a hereditary form of amyloidosis, with an autosomal recessive transmission mechanism. This type of amyloidosis affects people belonging to certain ethnic groups living along the coast of the Mediterranean Sea (Sephardic Jews, Greeks, Arabs, Armenians). There are varieties of amyloidosis characteristic of a particular geographic area: “Portuguese amyloidosis” (with predominant damage to the nerves of the lower extremities), “American amyloidosis” (with predominant damage to the nerves of the upper extremities), familial nephropathic amyloidosis, or “English amyloidosis” that occurs with symptoms of urticaria deafness and fever.
AH-amyloidosis (hemodialisis-related) is observed only in patients undergoing hemodialysis treatment. The pathogenesis is associated with the fact that, normally, kidney-utilized beta-2-microglobulin MHC class I, is not filtered in hemodialysis and accumulates in the body.
AE-amyloidosis is a form of local amyloidosis that develops in some tumors, for example, in the medullary carcinoma of the C-cells of the thyroid gland. In this case, the precursor of amyloid are pathological fragments of calcitonin.
Finnish-type amyloidosis is a rare type of amyloidosis caused by a mutation of the GSN gene encoding the Jelsolin protein.
Symptoms of Amyloidosis of the Esophagus
Dysphagia is characteristic when swallowing solid and dry food, especially when lying down, burping. Complications: amyloid ulcers of the esophagus and esophageal bleeding.
Diagnosis of Amyloidosis of the Esophagus
X-ray examination of the esophagus is hypotonic, peristalsis is weakened, and when examining the patient in a horizontal position, the barium suspension lingers long in the esophagus.
Treatment of amyloidosis of the esophagus
General principles for the treatment of amyloidosis
- Home mode, with the exception of severe conditions (severe heart failure, chronic renal failure)
- Patients with amyloidosis show a prolonged (1.5-2 years) reception of a raw liver (100-120 g/day)
- Restriction of protein and salt intake in patients with chronic renal failure
- Limiting salt to patients with heart failure
- In secondary amyloidosis, treatment of the underlying disease (tuberculosis, osteomyelitis, empyema, etc.), after the cure of which the manifestations of amyloidosis often disappear
- Transfer of a patient with dialysis amyloidosis to peritoneal dialysis
- When intestinal amyloidosis occurs with persistent diarrhea – astringents (bismuth subnitrate, adsorbents)
- In primary amyloidosis in the initial stages of the process – chloroquine 0.25 g 1 r/day for a long time, a combination of melphalan and prednisolone, melphalan, prednisolone and colchicine or only colchicine
- In secondary amyloidosis, specific treatment of the underlying disease
- With familial amyloidosis – colchicine (0.6 mg 2-3 r/day)
- Symptomatic therapy: vitamins, diuretics, drugs that reduce pressure, plasma transfusions, etc.
Surgical treatment of amyloidosis
- Removal of the spleen may improve the condition due to a decrease in the amount of amyloid produced in the body.