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Intestinal amyloidosis
Last reviewed: 07.07.2025
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Intestinal amyloidosis is a disease of the intestine (an independent disease or “second disease”) caused by the deposition of amyloid in its tissues.
In amyloidosis, the entire gastrointestinal tract can be affected, but the most significant amyloid deposition occurs in the small intestine.
The most well-known clinical type of intestinal amyloidosis is amyloidosis as a complication of many diseases of infectious, immune-inflammatory nature - secondary amyloidosis, in which the precursor of the protein of amyloid fibrils - the SAA protein - circulates in the blood. The same precursor serves as the basis for amyloidosis in periodic disease. Secondary amyloidosis and amyloidosis in periodic (hereditary) disease are combined into the AL-amyloidosis group. Intestinal amyloidosis can also be a manifestation of an independent nosological form of unknown nature (idiopathic, primary amyloidosis), or amyloidosis in chronic lymphatic paraproteinemic leukemia, primarily myeloma disease. In these cases, we are talking about AL-amyloidosis, in which amyloid fibrils build light chains of immunoglobulins circulating in the blood. Small intestine damage in secondary amyloidosis, according to clinical data, is observed in 40%, and according to pathological data - in 64% of patients, in primary amyloidosis - in 30-53 and 60-80% of patients, respectively. The large intestine is involved in the process somewhat less frequently: according to clinical data - in 30-55% of patients, according to section data - in 40-45% of patients. Information on the frequency of intestinal damage in hereditary (periodic disease) amyloidosis is contradictory.
It should be remembered that intestinal lesions occur mainly in forms of generalized amyloidosis (mainly AA and AL amyloidosis). Local tumor-like intestinal amyloidosis is very rare. When clinical manifestations of intestinal amyloidosis dominate, it is called the enteropathic type of amyloidosis.
Etiology and pathogenesis. The cause of amyloidosis, including intestinal amyloidosis, is not clear. The mechanism of amyloid formation can be considered disclosed only in AA and AL amyloidosis, i.e., those forms of generalized amyloidosis in which the intestine is most often affected.
Causes and pathogenesis of intestinal amyloidosis
Clinical picture
The gastrointestinal tract is affected throughout its entire length in amyloidosis. Macroglossia (significant enlargement of 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 intestinal amyloidosis
Diagnostics
The following signs may help in diagnosing intestinal amyloidosis:
- The presence of an underlying disease that leads to the development of intestinal amyloidosis (tuberculosis, bronchiectasis, rheumatoid arthritis, etc.).
- Persistent diarrhea resistant to therapy with antibacterial, astringent, adsorbent and fixing agents (amyloidosis with predominant damage to the small intestine).
- Clinical picture of malabsorption syndrome (characteristic of amyloidosis with predominant damage to the small intestine).
Diagnosis of intestinal amyloidosis
Treatment of intestinal amyloidosis. In amyloidosis, including intestinal amyloidosis, a complex of medications is recommended that affect the main links in the pathogenesis of the disease.
In order to influence the intracellular synthesis of amyloid protein, derivatives of 4-aminoquinoline (chloroquine, delagyl, plaquenil), corticosteroid hormones in small and medium doses, colchicine, immunostimulants: T- and B-activin, levamisole are prescribed.
Treatment of intestinal amyloidosis
Prevention of secondary amyloidosis is the prevention of chronic purulent-inflammatory, autoimmune and tumor diseases from the group of paraproteinemic leukemia.
The prognosis for intestinal amyloidosis is unfavorable, especially when malabsorption syndrome occurs, as well as such serious complications as bleeding and intestinal perforation. Involvement of the kidneys in the pathological process aggravates the prognosis. At the same time, the possibility of amyloid resorption in secondary amyloidosis against the background of colchicine treatment makes the prognosis for this form of the disease more favorable.
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