Amyloidosis - what is it? Amyloidosis: causes, symptoms, treatment, diagnosis

Amyloidosis - what is it? This disease, caused by a violation of protein metabolism, in which the formation and deposition in various tissues and organs of a specific protein-polysaccharide substance - amyloid.

Development of the disease

Amyloidosis develops (what is it - we already found out) in the violation of protein synthesis in the reticulo-endothelial system. In the blood plasma, an accumulation of abnormal proteins occurs. These proteins are essentially autoantigens and cause, by analogy with allergy, the formation of autoantibodies.

These antibodies with antigens then react and the coarsely dispersed proteins precipitate. This is the formation of amyloid. This substance settles on the vascular walls and various organs. Gradually accumulating, amyloid leads to the death of the organ.

Types of amyloidosis. Causes

There are several types of amyloidosis. The causes of the development of the disease directly depend on what kind of amyloidosis. What it is? Classification is carried out depending on the main protein, which consists of amyloid fibrils. Below are the types of this disease.

1. Primary amyloidosis (AL-amyloidosis). When it develops, anomalous light chains of immunoglobulins appear in the blood plasma, which are able to settle in various body tissues. In the same way, plasma cells change in myeloma, Waldenstrom's macroglobulinemia, monoclonal hypergammaglobulinemia.
2. Secondary amyloidosis (AA-amyloidosis). In this case, excessive release of the liver protein alpha-globulin occurs. This is an acute phase protein that is synthesized in a chronic inflammatory process. This is possible with various diseases, for example, with rheumatoid arthritis, malaria, bronchiectasis, osteomyelitis, leprosy, tuberculosis.
3. Family amyloidosis (AF-amyloidosis). This is a hereditary form of the disease with an autosomal recessive inheritance mechanism. It is also called the Mediterranean intermittent fever or familial paroxysmal polyserositis. This disease is expressed by attacks of a fever, occurrence of pains in a stomach, rashes on a skin, an arthritis and pleurisy.
4. Dialysis amyloidosis (AH-amyloidosis). It is connected with the fact that the protein beta-2-microglobulin MHC in healthy people is utilized by the kidneys, and under hemodialysis it is not filtered, and therefore its accumulation takes place in the body.
5. AE-amyloidosis. It develops in some forms of cancer, for example, the thyroid gland.
6. Aging amyloidosis.

Symptoms

When diagnosed as "amyloidosis," the symptoms depend on the location of the deposits. If the gastrointestinal tract is affected, an increased tongue, impaired swallowing function, constipation or diarrhea may occur. Sometimes amyloid tumoral deposits in the intestine or stomach are possible.

Amyloidosis of the intestine is accompanied by a sense of severity and discomfort, there may be mild pain in the abdomen. If the pancreas is affected, then there is the same symptomatology as with pancreatitis. If liver damage is observed, it increases, nausea, eructation, vomiting, jaundice.

Amyloidosis of the respiratory system is manifested as follows:

• A hoarse voice;
• Symptomatology of bronchitis;
• Pulmonary tumor-like amyloidosis.

With amyloidosis of the nervous system, the following symptoms can occur:

• Sensations of tingling or burning in the extremities, a feeling of numbness (peripheral polyneuropathy);
• Headaches, dizziness;
• Sphincter disorders (incontinence, feces).

Amyloidosis - what is it, the reasons for its occurrence and the symptoms we have considered. Now we will understand how this disease is diagnosed and what methods of its treatment exist.

Diagnostics

With such a disease as amyloidosis, the diagnosis is complex. Laboratory and hardware studies are assigned.

At laboratory researches in the general or common analysis of a blood increase of an ESR, leukocytes and depression of thrombocytes is observed. In the general analysis of urine there is a protein, in the sediment - cylinders, leukocytes and erythrocytes. In the coprogram there is a large amount of starch, fat and muscle fibers. In the biochemistry of blood with liver damage, there is an increased content of cholesterol, bilirubin, alkaline phosphatase.

In primary amyloidosis in urine and blood plasma, a high content of amyloid is found. At secondary during laboratory researches find out signs of a chronic inflammatory process.

Also carry out other diagnostic measures:

• X-ray examination;
• Echocardiography (in case of suspected heart disease);
• Functional tests with colorants;
• Biopsy of organs.

Treatment

With this disease, outpatient treatment is provided. Amyloidosis, in which severe conditions are observed, for example, in chronic renal failure or severe heart failure, are treated in a hospital.

At a primary amyloidosis at an initial stage such preparations, as "Chloroquine", "Melfalan", "Prednisolonum", "Kolkhitsin" are appointed.

In secondary amyloidosis, the underlying disease is treated, for example, osteomyelitis, tuberculosis, pleural empyema, etc. Often after its cure all symptomatology of amyloidosis disappears.

If the disease develops as a result of kidney hemodialysis, then this patient is transferred to peritoneal dialysis.

In case of diarrhea, astringent medications, for example, "Bismuth Subnitrate" or adsorptive agents, are used.

Apply also symptomatic treatment:

• Drugs that reduce blood pressure;
• Vitamins, diuretics;
• Transfusion of plasma, etc.

In addition, surgical treatment can also be used. Amyloidosis of the spleen can retreat after removal of the organ. In most cases this leads to an improvement in the condition of the patients and a decrease in the formation of amyloid.

Food

In case of amyloidosis, diet is required. With the development of chronic kidney failure should limit the consumption of salt and protein products, such as meat, fish, eggs. If chronic heart failure develops , salt, smoked and pickled foods should be excluded from the diet.

Amyloidosis of the heart

This disease is also called amyloid cardiopathy. In its development, amyloid deposition can occur in the myocardium, pericardium, endocardium or on the walls of the aorta and coronary vessels. The cause of this defeat of the heart can serve as primary amyloidosis, secondary or familial. Often, amyloidosis of the heart is not an isolated disease, and it develops in parallel with the amyloidosis of the lungs, kidneys, intestines or spleen.

Symptoms of amyloidosis of the heart

Often the symptomatology of this disease is similar to hypertrophic cardiopathy or ischemic heart disease. At the initial stage, the symptoms are not clearly expressed. Irritability and fatigue, some weight loss, swelling of the tissues and dizziness can be observed.

A sharp deterioration usually occurs after any stressful situations or respiratory infection. After that, usually there are heart pains like angina pectoris, arrhythmias, pronounced swelling, shortness of breath, enlarged liver. Blood pressure is usually low.

The disease progresses rapidly and its distinctive feature is resistance to treatment. In severe cases, patients may have ascites (fluid accumulation in the abdominal cavity) or pericardial effusion. Due to amyloid infiltrates, weakness of the sinus node and bradycardia develop. This can lead to sudden death.

Forecast

With amyloidosis, the prognosis is unfavorable. Heart failure in this disease is steadily progressing, and the lethal outcome is inevitable. In Russia there are no specialized centers dealing with this problem.