Amyloidosis: symptoms, treatment, causes, diagnosis

More than a hundred-year history of research accompanies, and to this day the common disease - amyloidosis. It is manifested by deposition in tissues of protein-polysaccharide compounds - amyloid.

What is it? The disease is caused by a violation of protein metabolism, as a result of which, amyloid dystrophy causes a change in the size of the organ, the formation of woody density and the acquisition of a greasy or waxy body.

Pathologies that are at risk for amyloidosis are very diverse. The most common:

• tuberculosis lesions;
  
• violent suppuration in inflammatory processes of bone;
  
• chronic lung abscess;
  
• systemic connective tissue diseases;
  
• chronic bronchial suppuration;
  
• for malignant diseases of lymphoid tissue and malignant tumors of various etiologies;
  
• chronic diseases of the colonic mucosa;
  
• for modular thyroid cancer;
  
• with endocarditis;parenchyma of the kidney and many other diseases.

There is no generally accepted classification of amyloidosis. Conditionally, for causative factors, it is customary to allocate:

1. 1) Primary - idiopathic form. Refers to the bronchopulmonary system - the parenchyma of the lungs, the walls of the vessels, the mucous membrane are affected.
   
2. 2) Secondary - acquired form. It develops as a complication of background diseases. Against the background of chronic infections of purulent-destructive nature, malignant tumors, lymphatic leukemia.
   
3. 3) Family, hereditary form. The result of the protein mutation is transthyretin in the male line.
   
4. 4) Senile amyloidosis is an independent form. Occurs with Alzheimer's disease, heart vessels, brain vessels and aorta are affected. The basis is the age( involution) violation of protein metabolism.
   
5. 5) Local tumor-like form. Figured as a stand-alone.a certain disease, on the basis of clinical manifestations.

Depending on the greatest development in various organs of fibrillar proteins - amyloids, the most common type is nephrotic.

Symptoms of amyloidosis of the kidneys

The presence of nephrotic symptoms due to prolonged course of chronic diseases is the most reliable sign of reactive, secondary amyloidosis of the kidneys.

There are cases of formation of amyloid deposits in the tissues of the kidneys in the primary form of the disease. In such a situation rapid development of nephrotic syndrome is noted. But, as a rule, patients die before the syndrome develops.

The main symptoms of nephropathy preceding amyloidosis of the kidneys are expressed:

• by gradual or very rapid development of edema on the entire subcutaneous surface;
  
• ascites, which causes the violation of the water-salt balance;
  
• appears shortness of breath;
  
• major changes in cardiac activity;
  
• sudden symptoms of azotemia;
  
• development of an acute form of hypertension.

All this symptomatology, especially swelling, develops quite a long period. Therefore, the course of the disease determines the stages of the disease.

Symptoms of amyloidosis

Amyloidosis, and its characteristic clinical symptoms will directly depend on the stage at which the process is located.

1) In the latent, asymptomatic stage of the disease, the localization of the amyloid precipitate is noted in the intermediate layer of the navicular( large, superficial) cells. Has a five-year milestone. During this time, in the presence of secondary, reactive amyloidosis, there are signs of different background diseases - purulent abscesses in the lungs, manifestations of tuberculous lesions or joint pathologies.

2) The initial albuminuric stage of the secondary form of the disease - the amyloid deposit is localized between the capillaries of the renal glomeruli, in their loops and kidney vessels. There is a profound damage to the glomerular damage of the kidney filter, they become larger and acquire a dense consistency. The duration of the stage is determined by thirteen-year.

3) Stage of nephrotic, puffiness - presence of amyloid is noted in all parts of the kidney. It affects the medulla of the kidney, there is tissue sclerosis. The kidney becomes large and greasy. Characteristic of all the signs of nephropathy with extensive swelling. The disease develops over a six-year period. Adding to nephrotic symptoms is intestinal involvement, enlarged spleen, and lymph nodes.

4) With uremic, terminal, stage - the development of the disease occurs on the basis of hypotension and nephrosis. Characteristic of various renal pathologies - a change in the type and consistency of the kidney.

The presence of an amyloid deposit on the kidneys does not mean that they do not exist in other organs.

1. 1) Amyloid deposition in the skin is manifested by skin reddening and small wrinkles on it.
   
2. 2) Presence of deposits in the lymphatic system is manifested by enlarged lymph nodes;
   
3. 3) When the digestive tract is affected, there are noted - difficulty eating when swallowing, swelling of the tongue, hepatomegaly and indigestion are noted.
   
4. 4) The nervous system, peppered with amyloid, provokes the development of dementia;
   
5. 5) Difficulty in breathing and shortness of breath is a characteristic symptom of the presence of sediments that affect the respiratory tract.
   
6. 6) Amyloid deposition on the heart muscle leads to a violation of the normal heart rhythm, cardiomegaly, which can provoke cardiac arrest and lead to death;

Causes of amyloidosis

The main cause of amyloidosis for a hundred years, and to the end and not studied. There are only scientifically formulated versions and assumptions. These include:

• Immunological Theory of Development;
  
• result of a protein mutation;
  
• a violation of protein metabolism, as a result of which accumulation of amyloid occurs in the vascular walls, glands and blood plasma;
  
• inflammatory processes, infectious and chronic diseases, transferred bone marrow diseases.

Even after the death of the patient, the amyloid is not subject to decomposition for a long time.

Diagnosis of amyloidosis

It is difficult to diagnose the disease at an early stage, since during this period amyloidosis often has an asymptomatic form. Suspicion of amyloid deposition is possible by the results of a urine test for microalbuminuria, which is an indicator of the early kidney damage process, and diagnoses the initial, early pathology of the vessels.

The index of renal amyloidosis can be an analysis of the concentration of Cystatin in the blood, which determines the rate of glomerular renal filtration. For advanced diagnostics, a number of special tests and tests are used:

1. 1) Analysis for clinical parameters of blood and urine.
   
2. 2) Analysis of the determination of daily( proteinuria) loss of protein in the urine.
   
3. 3) Determination of the cause of the increased composition in the urine of erythrocytes, leukocytes and cylinders in the Nechiporenko analysis.
   
4. 4) Urine analysis according to Zimnitsky - determination of the presence of hormones.
   
5. 5) Daily urine analysis for determination of creatinine level - Rebergh's test.
   
6. 6) Determination of acids and protein fractions in the blood.
   
7. 7) Study of kidneys by radioisotope radiography and ultrasound.
   
8. 8) Pathognomonic test using methylene blue to detect amyloids.
   
9. 9) Study of the hypoderma and tissue of the kidneys using the method of sampling bioblast tissues;

With a biopsy of the muscle tissue of the tongue and gums, the analysis can become more significant and useful in the idiopathic form of the disease with amyloid precipitate in the peroxygenase space.

Treatment of amyloidosis

Unfortunately, modern treatment is not able to guarantee a complete cure for amyloidosis. Therapeutic measures are aimed at curing background diseases and their manifestations, controlling the situation in order to avoid pathological processes and using medications to improve the overall condition of the patient.

In time, the revealed pathology makes it possible to stop the development of the disease, reducing the harmful effect of amyloid. Stabilize and maintain the patient's condition by applying an individually formulated curative program:

• getting rid of the symptoms of background pathologies.
  
• prevention of nephrotic manifestations.
  
• control of salt intake.
  
• administration of thiazide diuretics and thiazide diuretics.
  
• transfusion of erythrocyte masses is performed to eliminate tissue hypoxia due to anemia.
  
• is assigned intensive therapy to correct water-electrolyte disorders.
  
• is an individual diet.

Drug treatment for amyloidosis

For the retention of the pathological process within the progression of the disease, it is recommended to take medications, according to an individually designed schedule.

1. 1) Colchicine is a herbal preparation that helps to lower the migration of leukocytes into inflamed foci and to inhibit the phagocytosis of salt microcrystals.
   
2. 2) Unithiol solution - is prescribed for functional liver disorders;
   
3. 3) Dimethyl sulfoxide solution - an anti-inflammatory and analgesic preparation is added to the fruit juice.
   
4. 4) Delagil - an immunostimulating drug, is effective in the treatment of infections.

To prevent the development of symptoms prescribed chemotherapy course. In serious cases, donor stem cells are transplanted or the donor kidney is completely replaced.

To reduce amyloid deposits, in certain situations, it is advisable to remove the spleen. In the early treatment of the secondary form of the disease, it is sometimes sufficient to cure the background disease so that the symptoms of amyloidosis disappear.

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