Amyloidosis - what is it? This is a disease caused by a violation of protein metabolism, in which the formation and deposition in a variety of tissues and organs of a specific protein-polysaccharide substance - amyloid.
Disease development
Amyloidosis develops (what it is - we have already found out) with a violation of protein synthesis in the reticuloendothelial system. In the blood plasma, an abnormal protein accumulates. These proteins are essentially autoantigens and cause the formation of autoantibodies by analogy with allergies.
Then these antibodies with antigens react and coarsely dispersed proteins precipitate. This is the formation of amyloid. This substance settles on the vascular walls and various organs. Accumulating gradually, the 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 type of amyloidosis. What it is? The classification is carried out depending on the main protein of which amyloid fibrils are composed. Below are the types of this disease.
- Primary amyloidosis (AL-amyloidosis). With its development, abnormal light chains of immunoglobulins appear in the blood plasma, which are able to settle in a variety of body tissues. In the same way, plasma cells change with myeloma, Waldenstrom macroglobulinemia, monoclonal hypergammaglobulinemia.
- Secondary amyloidosis (AA amyloidosis). In this case, excessive secretion of alpha-globulin protein by the liver 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.
- Familial amyloidosis (AF-amyloidosis). This is a hereditary form of the disease with an autosomal recessive inheritance mechanism. It is also called Mediterranean intermittent fever or familial paroxysmal polyserositis. This disease is expressed by bouts of fever, the occurrence of abdominal pain, skin rashes, arthritis and pleurisy.
- Dialysis amyloidosis (AH-amyloidosis). It is associated with the fact that beta-2-microglobulin MHC protein in healthy people is utilized by the kidneys, and during hemodialysis it is not filtered, and therefore it accumulates in the body.
- AE amyloidosis. It develops in some forms of cancer, for example, the thyroid gland.
- Senile amyloidosis.
Symptoms
With a diagnosis of amyloidosis, the symptoms depend on the location of the deposits. In case of damage to the gastrointestinal tract, an enlarged tongue, impaired swallowing function, constipation or diarrhea can be observed. Amyloid tumor-like deposits in the intestines or stomach are sometimes possible.
Intestinal amyloidosis is accompanied by a feeling of heaviness and discomfort, there may be moderate pain in the abdomen. If the pancreas is affected, then the same symptoms are present as with pancreatitis. With damage to the liver, its increase is observed, nausea, belching, attacks of vomiting, jaundice appear.
Amyloidosis of the respiratory system is manifested as follows:
- a hoarse voice;
- symptoms of bronchitis;
- pulmonary tumorous amyloidosis.
With amyloidosis of the nervous system, the following symptoms can be observed:
- tingling or burning sensations in the limbs, numbness (peripheral polyneuropathy);
- headaches, dizziness;
- sphincter disorders (urinary incontinence, feces).
Amyloidosis - what is it, the causes of its occurrence and symptoms we examined. Now let's see how this disease is diagnosed and what methods of its treatment exist.
Diagnostics
With a disease such as amyloidosis, the diagnosis is complex. Laboratory and hardware studies are assigned.
In laboratory tests, in an overall blood test, an increase in ESR, white blood cells and a decrease in platelets is observed. In the general analysis of urine, protein is present, in the sediment - cylinders, white blood cells and red blood cells. The coprogram contains a large amount of starch, fat and muscle fibers. In blood biochemistry with liver damage, an increased content of cholesterol, bilirubin, alkaline phosphatase is detected.
With primary amyloidosis, a high amyloid content is found in urine and blood plasma. When secondary during laboratory tests, signs of a chronic inflammatory process are found.
Also carry out other diagnostic measures:
- X-ray examination;
- echocardiography (in case of suspected heart damage);
- functional tests with dyes;
- organ biopsy.
Treatment
With this disease, outpatient treatment is performed. Amyloidosis, in which severe conditions are observed, for example, with chronic renal failure or severe heart failure, is treated in a hospital.
With primary amyloidosis, drugs such as Chloroquine, Melphalan, Prednisolone, and Colchicine are prescribed at the initial stage.
With secondary amyloidosis, the underlying disease is treated, for example, osteomyelitis, tuberculosis, pleural empyema, etc. Often, after its cure, all the symptoms of amyloidosis disappear.
If the disease develops as a result of renal hemodialysis, then such a patient is transferred to peritoneal dialysis.
In case of diarrhea, astringent drugs are used, for example, Bismuth Subnitrate or adsorbing agents.
Symptomatic treatment is also used:
- drugs that reduce blood pressure;
- vitamins, diuretics;
- plasma transfusion, etc.
In addition, surgical treatment may also be used. Spleen amyloidosis may recede after organ removal. In most cases, this leads to an improvement in the condition of patients and a decrease in the formation of amyloid.
Nutrition
With amyloidosis, you must constantly follow a diet. With the development of chronic renal failure, you should limit the use 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. With its development, amyloid deposition can occur in the myocardium, pericardium, endocardium or on the walls of the aorta and coronary vessels. The cause of such heart damage can be primary amyloidosis, secondary or familial. Often, amyloidosis of the heart is not an isolated disease, and it develops in parallel with amyloidosis of the lungs, kidneys, intestines or spleen.
Symptoms of Amyloidosis of the Heart
Often the symptoms of this disease are similar to hypertrophic cardiopathy or coronary 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 this, pain in the heart usually appears as angina pectoris, arrhythmias, severe swelling, shortness of breath, enlarged liver. Blood pressure is usually low.
The disease progresses rapidly and its hallmark is resistance (resistance) to the therapy. In severe cases, patients may experience ascites (fluid accumulation in the abdominal cavity) or pericardial effusion. Due to amyloid infiltrates, sinus node weakness and bradycardia develop. This can lead to sudden death.
Forecast
With amyloidosis of the heart, the prognosis is poor. Heart failure with this disease is steadily progressing, and a fatal outcome is inevitable. In Russia there are no specialized centers dealing with this problem.