Amyloidosis of the intestine
Last reviewed: 23.04.2024
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Amyloidosis of the intestine is a disease of the intestine (an independent disease or "second disease"), caused by the deposition of amyloid in its tissues.
Amyloidosis can affect the entire gastrointestinal tract, but the most significant deposition of amyloid occurs in the small intestine.
The most known clinical variant of amyloidosis of the intestine is amyloidosis as a complication of many infectious, immunoinflammatory diseases - secondary amyloidosis, in which the precursor of amyloid fibril protein-SAA protein circulates in the blood. The same precursor serves as the basis of amyloidosis with a periodic illness. Secondary amyloidosis and amyloidosis with periodic (hereditary) disease are combined into the AL-amyloidosis group. Amyloidosis of the intestine can also be a manifestation of an independent nosological form of an unknown nature (idiopathic, primary amyloidosis), or amyloidosis in chronic lymphatic paraproteinemic leukemia, primarily myeloma. In these cases, we are talking about AL-amyloidosis, in which fibrils of amyloid build blood-circulating light chains of immunoglobulins. The defeat of the small intestine in secondary amyloidosis, according to clinical data, is observed in 40%, and in pathoanatomical - in 64% of patients, in primary amyloidosis - in 30-53 and 60-80% of patients, respectively. The large intestine is involved in the process more rarely: according to clinical data, 30-55% of patients, according to sections - in 40-45% of patients. Information on the incidence of bowel disease in hereditary (recurrent disease) amyloidosis is contradictory.
It should be remembered that intestinal lesions occur mainly in the forms of generalized amyloidosis (predominantly AA- and AL-amyloidosis). Very rarely occurs local tumoral amyloidosis of the intestine. When clinical manifestations of amyloidosis of the intestine dominate, they speak of the enteropathic type of amyloidosis.
Etiology and pathogenesis. The cause of amyloidosis, including the intestine, is not clear. The mechanism of amyloid formation can be considered open only with AA- and AL-amyloidosis, that is, those forms of generalized amyloidosis, in which the intestine is most often affected.
Causes and pathogenesis of intestinal amyloidosis
Clinical picture
Gastrointestinal tract in amyloidosis is affected all over. Macroglossia (a significant increase in the tongue) is observed in 20-22% of patients, hepatomegaly and splenomegaly - in 50-80% of patients, the esophagus may be affected, sometimes there is a tumor-like lesion of the stomach.
Symptoms of amyloidosis of the intestine
Diagnostics
The following symptoms can help in the diagnosis of amyloidosis of the intestine:
- The presence of the underlying disease, which leads to the development of amyloidosis of the intestine (tuberculosis, bronchiectatic disease, rheumatoid arthritis, etc.).
- Persistent diarrhea resistant to antibacterial, astringent, absorbent and fixing agents (amyloidosis with predominant lesion of the small intestine).
- Clinical picture of malabsorption syndrome (typical for amyloidosis with predominant involvement of the small intestine).
Diagnosis of amyloidosis of the intestine
Treatment of amyloidosis of the intestine. In amyloidosis, including amyloidosis of the intestine, a complex of medications is recommended that have an effect on the main links in the pathogenesis of the disease.
In order to influence the intracellular synthesis of amyloid protein, 4-aminoquinoline derivatives (chloroquine, delagil, plaquenil), corticosteroid hormones in small and medium doses, colchicine, immunostimulants: T- and B-activin, levamisole, are prescribed.
Treatment of amyloidosis of the intestine
Prevention of secondary amyloidosis is the prevention of chronic purulent-inflammatory, autoimmune and neoplastic diseases from the paraproteinemic leukemia group.
The prognosis for amyloidosis of the intestine is unfavorable, especially when there is a syndrome of impaired absorption, as well as such formidable complications as bleeding and perforation of the intestine. Involvement in the pathological process of the kidneys burdens the prognosis. However, the possibility of resorption of amyloid in secondary amyloidosis on the background of treatment with colchicine makes a prognosis for this form of the disease more favorable.
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