Renal amyloidosis

Renal amyloidosis rarely develops, but this pathology should not be ignored. According to statistics, the disease affects 1 person out of 50-60 of the population. Often amyloidosis resembles the symptoms of glomerulonephritis or chronic pyelonephritis. It is possible to identify the disease only after all other pathologies of the urinary system are excluded. In order not to miss the beginning of the development of amyloidosis, you need to know its main symptoms and be able to recognize them in time.

Amyloidosis – what is it?

Amyloidosis – a group of diseases caused by a violation of protein metabolism: amyloid is formed and deposited in the tissues – a specific protein-polysaccharide complex. With amyloidosis, the work of various organs and tissues becomes difficult.

Amyloid is a substance that is practically not produced in a healthy human body. It has a complex structure, represented by proteins and sugars. It does not contain pathogenic flora, such as viruses or bacteria. Therefore, the body does not perceive an increase in the concentration of amyloid as a pathology and does not give an appropriate reaction to it. The fight is simply non-existent. While an excess of amyloid negatively affects the state of health.

Scientists suggest that amyloidosis is a hereditary disease that develops due to a malfunction in the immune system. Although the exact causes of amyloidosis have not been established to date.

Amyloid is harmful to the kidneys. Accumulating in them, it destroys their tissues, leading to a metabolic disorder. The pathological process develops slowly, the symptoms are subtle, or absent altogether. Over time, the kidneys cease to cope with their functions, which leads to fluid retention in the body. In addition, harmful compounds remain in it, such as: urea, uric acid and creatinine.

Causes of amyloidosis

Depending on the cause that provoked amyloidosis, there are 3 types of it:

• Primary amyloidosis. In this case, the disease is inherited. Chromosomal abnormalities cause cells of the immune system to produce abnormal protein compounds that the body transforms into amyloid.

• Secondary amyloidosis. The cause of this type of disease is another disorder in the body. Chronic pathology leads to a malfunction of immune cells.

• Aging amyloidosis. As we age, the body ceases to function fully. This applies to all systems, including immunity. The number of leukocytes in the elderly remains at the same level as in young people, but their metabolism undergoes certain changes. Therefore, pathological compounds, which include amyloid, begin to be produced in the body.

All of these putative causes for the development of amyloidosis made it possible to identify people who are at an increased risk for developing amyloidosis:

• Persons with a family history of amyloidosis or other kidney pathologies of unclear etiology.

• People over 65.

• Persons with rheumatoid arthritis, Bechterew’s disease, SLE, cancerous neoplasms.

Such people need to monitor the work of the kidneys and, in case of any violations in their functioning, consult a doctor to rule out amyloidosis.

Amyloidosis symptoms

The disease develops over time. Symptoms increase gradually. More than 10 or even 20 years may pass from the moment the first signs of a violation appear to the appearance of severe symptoms. Despite such a slow progression of the pathology, it is possible to identify it too late to prescribe an effective therapy to the patient.

The first symptoms of amyloidosis of the kidneys

In the early stages of development, the pathology proceeds hidden. The first sign of amyloidosis is protein in the urine. The fact of his presence does not affect the well-being of a person, nothing hurts him, his blood pressure does not change.

The loss of protein in the urine does not pose a threat to the body, since there is very little of it. However, this clinical sign should alert the doctor, as it indicates a violation of the kidneys. As the disease progresses, this symptom will entail a whole cascade of pathological reactions.

Protein in the urine can be excreted for a long time, for 10-15 years. Proteinuria can be detected only after passing laboratory tests. Therefore, all persons who belong to the risk group need to give urine for analysis twice a year. To do this, just contact the clinic at the place of residence.

The main symptoms of progressive amyloidosis

As the pathology progresses, a significant amount of amyloid accumulates in the body, which leads to a disruption in its work. The kidneys are not able to filter and retain substances necessary for a person. Therefore, with urine per day, he will lose about 3 g of protein, with normal losses of 0,13 g / l. At the same time, edema begins to appear in patients. Their intensity varies, ranging from slight swelling of the face and ending with sweating of fluid into tissues and organs.

Other symptoms of amyloidosis:

• Urination becomes more frequent, becomes plentiful. The kidney filter is not able to perform its functions, so up to 10 liters of fluid are excreted in the urine per day. This disorder is called kidney diabetes. Although the loss of fluid is significant, this does not allow you to cope with edema, since a certain part of it leaves the blood vessels and enters the tissues.

• Violation of blood pressure. With amyloidosis, it can increase or decrease. It is impossible to predict what kind of failure will happen in a particular patient. However, most people suffer from low blood pressure.

• Urine takes on an unusual color. She becomes reddish. This symptom indicates that the kidneys are being destroyed.

• A set of extra pounds. Weight gain due to swelling. In addition, disturbances in the functioning of the kidneys lead to a failure in the metabolism of fats. The patient has elevated levels of cholesterol, triglycerides and lipoproteins. Fat begins to be deposited in the tissues, the figure on the scales creeps up.

Doctors call such a symptom complex amyloid-lipoid nephrosis. The duration of this stage is 6 years (averaged values). If during this period the pathology is detected and the person begins to receive treatment, then the prognosis is more or less favorable. Otherwise, the disease will go into the stage of irreversible changes.

irreversible kidney damage

This stage is characterized by loss of kidney function. In addition to the fact that they stop passing protein, the organs lead to the retention of toxins (uric acid, urea, bilirubin, creatinine, etc.).

Symptoms that come to the fore:

• Marked weakness.

• Deterioration of attention.

• Headache.

• Dizziness.

• Loss of consciousness.

• During this period, the patient may fall into a coma.

The patient is diagnosed with chronic renal failure. At the same time, blood pressure will be reduced, and symptoms of a violation in the work of other internal organs will also appear, since amyloid will also begin to accumulate in them.

Such violations include:

• Skin lesion. Translucent nodules appear on the face, neck, armpits and popliteal fossae. The skin under the eyes will be saturated with blood. It becomes dry in the wrist area.

• Heart failure. The patient develops shortness of breath, which occurs after physical exertion. He is also worried about chest pains, localized on the left side.

• Damage to the digestive tract. The tongue increases in size, teeth marks are visible on it. A few hours after eating, pain appears in the right side of the abdomen. The feces become black, shiny and extremely fetid. This sign indicates gastric or intestinal bleeding. The chair may be absent for 3 days or more.

• Joint damage. In the morning the patient experiences stiffness in the limbs. It becomes less intense after movements.

• Damage to the nervous system. Feet and hands lose sensitivity, the skin on them becomes less elastic, begins to peel off.

Even one of these symptoms is a reason to suspect amyloidosis.

Diagnostics

To identify the disease, it will take effort, especially since in the early stages of its development it does not give any symptoms. The tests that the patient will need to take are described in the table.

Laboratory diagnostics does not allow diagnosing amyloidosis.

To confirm it, you need to conduct the following tests:

• Subcutaneous tissue puncture. The collected material is examined for the detection of amyloid in it. To do this, it is stained with special solutions and examined under a microscope. The presence of amyloid allows the diagnosis of amyloidosis.

• Kidney puncture. This procedure is analogous to a subcutaneous tissue puncture, but the material is taken directly from the internal organ itself.

In addition, the patient is referred for an ultrasound of the kidneys, CT or MRI. These techniques will not make it possible to detect amyloid, but will allow you to assess the degree of damage to the urinary system.

Treatment of amyloidosis of the kidneys

Therapy of amyloidosis of the kidneys is difficult, since the disease is difficult to correct. It is impossible to remove amyloid from organs by 100% with the help of drugs or physiotherapy. Provided that the treatment was started early, it is possible to reduce the rate of progression of the disease and prevent serious damage to the kidneys.

Compliance with a diet. Dietary nutrition is the most important condition for the treatment of amyloidosis.

To reduce the production of amyloid in the body and relieve the burden on the kidneys, it is necessary to exclude or limit substances such as:

• Salt. You can add salt to dishes during cooking, but you should not add salt to already cooked food. Canned food and salinity are excluded.

• Casein. This substance is present in dairy products. It is also found in mixtures for the nutrition of athletes.

• Beef and veal.

The menu may include cereals, vegetables, lamb, chicken. Flour products are excluded, which is especially important for overweight people.

Taking medications:

There is no single treatment regimen for amyloidosis. Therefore, the doctor can adjust the therapy at his own discretion.

Kidney transplant. This method is rarely implemented in practice. It is resorted to only in extreme cases, when there is a real threat to the patient’s life. In addition, finding a suitable donor in Russia is quite problematic. Therefore, such an operation is rare.

Forecast

If treatment is started on time, the progression of the disease can be delayed by 30-40 years. In general, the quality of life of patients with amyloidosis does not significantly deteriorate. Although from time to time they still experience some discomfort.

The death of patients from amyloidosis occurs only when the kidney undergoes irreversible changes. This occurs with the rapid progression of the pathology, or with its late detection.

Answers to popular questions

• What is the effect of Colchicine in the treatment of amyloidosis? Now this drug is rarely used, as it leads to the development of severe side effects. However, its effectiveness in the treatment of amyloidosis is proven. Therefore, if the doctor considers it necessary to use Colchicine, then you should heed his advice.

• What are the risks of hereditary transmission of the disease? Such risks exist. They increase if amyloidosis is traced in history in several family members. To reduce them, before conception, it is necessary to reduce the intensity of the pathological process as much as possible.

• Are there measures to prevent the development of amyloidosis? No such measures have been developed.

• Can the disease recur after a kidney transplant? Such a possibility exists, and it is quite high. However, about 15 years pass from the onset of the disease to the appearance of the first symptoms. If treatment is started on time, then this period increases by 2-3 times. Therefore, repeated kidney damage simply does not have time to develop again.