Amyloidosis - what is it? This is a 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.
Disease development
Amyloidosis develops (what it is - we have already found out) in violation of protein synthesis in the reticuloendothelial system. Abnormal proteins accumulate in the blood plasma. These proteins are essentially self-antigens and cause the formation of autoantibodies by analogy with allergies.
Then, these antibodies react with antigens and coarsely dispersed proteins precipitate. This is how amyloid is formed. This substance settles on the vascular walls and various organs. Gradually accumulating, amyloid leads to the death of the organ.
Types of amyloidosis. Reasons
There are several types of amyloidosis. The causes of the development of the disease directly depend on the type of amyloidosis. What it is? Classification is carried out depending on the main protein that makes up the amyloid fibrils. Below are the types ofdiseases.
- Primary amyloidosis (AL-amyloidosis). With its development, abnormal light chains of immunoglobulins appear in the blood plasma, which are able to settle in various tissues of the body. In the same way, plasma cells change in multiple myeloma, Waldenström's macroglobulinemia, monoclonal hypergammaglobulinemia.
- Secondary amyloidosis (AA-amyloidosis). In this case, there is an excess secretion of alpha-globulin protein by the liver. It is an acute phase protein that is synthesized during a chronic inflammatory process. This is possible in various diseases, such as rheumatoid arthritis, malaria, bronchiectasis, osteomyelitis, leprosy, tuberculosis.
- Family 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 attacks of fever, the occurrence of abdominal pain, skin rashes, arthritis and pleurisy.
- Dialysis amyloidosis (AH-amyloidosis). It is related to the fact that the protein beta-2-microglobulin MHC in he althy people is utilized by the kidneys, and during hemodialysis it is not filtered, and therefore it accumulates in the body.
- AE-amyloidosis. Develops in some forms of cancer, such as thyroid cancer.
- senile amyloidosis.
Symptoms
When diagnosed with amyloidosis, the symptoms depend on the location of the deposits. When defeatedof the gastrointestinal tract, an enlarged tongue, difficulty swallowing, constipation or diarrhea may be observed. Amyloid tumor-like deposits in the intestine 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. When the liver is damaged, its increase is observed, nausea, belching, bouts of vomiting, jaundice appear.
Respiratory amyloidosis manifests as follows:
- hoarse voice;
- symptoms of bronchitis;
- pulmonary amyloidosis tumor.
Nervous system amyloidosis may present with the following symptoms:
- tingling or burning sensation in limbs, numbness (peripheral polyneuropathy);
- headaches, dizziness;
- sphincter disorders (urinary incontinence, feces).
Amyloidosis - what it is, we have considered its causes and symptoms. Now let's figure out how this disease is diagnosed and what methods of its treatment exist.
Diagnosis
In a disease such as amyloidosis, the diagnosis is complex. Assigned laboratory and hardware research.
In laboratory studies in the general blood test, an increase in ESR, leukocytes and a decrease in platelets are observed. In the general analysis of urine there is protein, in the sediment there are cylinders, leukocytes and erythrocytes. In the coprogramthere is a large amount of starch, fat and muscle fibers. In the biochemistry of the blood with liver damage, an increased content of cholesterol, bilirubin, alkaline phosphatase is found.
In primary amyloidosis, a high content of amyloid is found in the urine and blood plasma. In the secondary during laboratory tests, signs of a chronic inflammatory process are found.
Also carry out other diagnostic measures:
- radiological examination;
- echocardiography (in case of suspected heart disease);
- functional tests with dyes;
- organ biopsy.
Treatment
This disease is treated as an outpatient. Amyloidosis in which there are severe conditions, such as chronic renal failure or severe heart failure, is treated in a hospital.
In primary amyloidosis, at the initial stage, drugs such as Chloroquine, Melphalan, Prednisolone, Colchicine are prescribed.
In 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 kidney hemodialysis, then such a patient is transferred to peritoneal dialysis.
Astringents such as "Bismuth Subnitrate" or adsorbents are used if diarrhea develops.
Symptomatic treatment is also used:
- drugs that reduce blood pressure;
- vitamins, diuretics;
- plasma transfusion, etc.
In addition, surgical treatment can be used. Amyloidosis of the spleen may recede after removal of the organ. In most cases, this leads to an improvement in the condition of patients and a decrease in the formation of amyloid.
Food
Amyloidosis requires a constant diet. With the development of chronic renal failure, the intake of s alt and protein foods such as meat, fish, and eggs should be limited. If chronic heart failure develops, s alt, smoked and pickled foods should be excluded from the diet.
Cardiac amyloidosis
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 damage to the heart may 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 cardiac amyloidosis
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, tissue swelling and dizziness may occur.
A sharp deterioration usually occurs after any stressful situations or a respiratory infection. After that, pain in the heart usually appears according to the type of angina pectoris, arrhythmias, pronounced edema, shortness of breath, enlargement of the liver. Blood pressure is usually low.
The disease progresses rapidly and its distinguishing feature is resistance (resistance) to ongoing therapy. In severe cases, patients may have ascites (fluid buildup in the abdomen) or pericardial effusion. Due to amyloid infiltrates, weakness of the sinus node and bradycardia develop. This can lead to sudden death.
Forecast
With amyloidosis of the heart, the prognosis is unfavorable. Heart failure in this disease is steadily progressing, and death is inevitable. There are no specialized centers in Russia dealing with this problem.
