One of the most common forms of amyloidosis is renal amyloidosis. In most cases, the disease develops as a complication of such serious pathologies as tuberculosis, syphilis, osteomyelitis. The defeat of the organ is a consequence of the underlying disease, which is recommended to identify and treat in a timely manner. Pathology appears as a result of an increase in the blood of amyloid (a complex protein), which settles on the walls of blood vessels and in the kidneys, as a result of which the organ's functionality is impaired. This disease proceeds against the background of nephrotic syndrome and goes into the development of chronic renal failure. In severe cases, a donor kidney transplant may be required.
Problem Description
Amyloidosis of the kidneys is a complication of systemic amyloidosis, which is characterized by the accumulation in the intercellular space of the kidneys of a glycoprotein substance - amyloid, which leads to a disorder of the organ.
Amyloid acts as a product of the synthesis of pathological proteins as a result of dysproteinemia, which occurs due to a chronic disease. This is due to autoimmune reactions. As a result of the development of a chronic disease, the decomposition products of toxins, leukocytes and tissues accumulate, which leads to the formation of antibodies, which upon reaction with antigens form amyloids. These amyloids are deposited in places where antibodies accumulate.
Secondary renal amyloidosis is of great importance in medical practice, since in most cases it leads to the formation of renal failure, in which the kidneys increase in size and have a smooth surface.
Epidemiology
There are differences in the prevalence of the disease among different world countries. In developed countries in Europe, the disease occurs in 0.7% of cases, and in Asia - up to 0.2%. This is due to the difference in the nutrition of people. In the latter case, people often eat food that contains a small amount of cholesterol and animal proteins. Cases of family pathology are most diagnosed in the countries of Spain and Portugal. Amyloidosis usually affects males between the ages of forty and fifty, but there are cases of the disease in children, including newborns.
Recently, the frequency of diagnosing pathology is growing. Especially often, the disease is accompanied by lung pathology, arthritis, ulcerative colitis, and cancerous tumors. Often, amyloidosis develops in those people who have had tuberculosis or purulent infections. Amyloidosis is also often diagnosed in drug addicts who inject drugs, which often provokes the development of suppuration.
Types of disease
In medicine, it is customary to distinguish the following types of pathology:
- The primary disease that appears as a result of the adhesion disorder of immunoglobulins, which change the composition of the blood, settling on the walls of blood vessels and internal organs. This pathology is difficult to diagnose. Since it has similar symptoms with other diseases.
- Secondary renal amyloidosis is due to chronic diseases: osteomyelitis, colitis, arthritis, sclerosis, tuberculosis or spondylitis. The disease often appears in old age.
All these types of amyloidosis occur and develop most often in childhood. The disease proceeds with periods of remission and relapse, which are accompanied by bouts of pain. Treatment in this case does not bring results.
Forms of the disease
In medicine, it is customary to distinguish several forms of the disease:
- An idiopathic form in which the causes and mechanisms of the disease are unknown.
- Family form, which is distinguished by a genetic predisposition to the appearance of abnormal proteins in the body. In medicine, it has been established that amyloidosis is transmitted through several generations. Typically, this phenomenon is observed among Jews and Arabs. The disease is accompanied by skin rashes, pain in the abdomen.
- The acquired form is characterized by the development of pathology as a result of immunological disorders.
- The senile form is characterized by a disorder of protein metabolism.
- The local form has unexplained causes of development. It is usually associated with endocrine organ tumors, diabetes, and Alzheimer's syndrome.
- A tumor-like form also develops for unexplained reasons. Doctors tend to claim that amyloidosis of this form develops due to some types of neoplasms.
Causes of amyloidosis
Amyloidosis of the kidneys in most cases, the causes are unknown. Many diseases can provoke the appearance of complications in the form of amyloidosis, but it can also act as an independent disease.
With primary pathology, it is not possible to establish the reasons for it. Sometimes the disease forms with multiple myeloma. In this case, in addition to the kidneys, the tongue, skin, heart, lungs, liver, intestines and spleen are included in the pathological process.
Secondary renal amyloidosis is formed due to chronic diseases of an infectious nature and abnormal conditions several years after their occurrence. Most often, the disease develops after a complication of pulmonary tuberculosis; it affects not only the kidneys, but also the vessels and lymph nodes. Senile amyloidosis is a sign of aging and occurs in 80% of people who are over eighty years old.
The risk group includes people who have diseases with damage to plasma cells, chronic inflammatory diseases, ankylosing spondylitis, rheumatoid arthritis, as well as Mediterranean fever.
Symptoms and signs of the disease
Renal amyloidosis symptoms may vary depending on the location of amyloid accumulations in other organs, the stage of the course of the disease, the degree of impaired activity of the affected organ, as well as the ailment that caused the appearance of the pathology.
The first signs of kidney damage are observed at an early stage of the development of the disease. In humans, the level of protein in the urine increases, weakness, anemia, impaired immunity, a disorder of the cardiovascular system, diarrhea appear. This disease provokes an increase in internal organs and the development of swelling. At a late stage of the disease, hypertension, anuria, and renal vein thrombosis are observed.
In medicine, the following symptoms of the manifestation of amyloidosis are recorded:
- impaired renal function, the development of renal failure;
- disorders of the activity of the digestive tract;
- damage to the nervous system;
- the development of arrhythmia and heart failure.
With amyloidosis, edema always develops. In some cases, renal vein thrombosis appears, pain syndrome, arrhythmia, dizziness, shortness of breath develop.
Stages of renal amyloidosis
During the disease, several stages are distinguished, each of which has its own manifestation of symptoms:
- The latent stage is characterized by an asymptomatic course of amyloidosis, signs of primary pathology may appear. The main sign of the latent period is the presence of minor proteinuria. This period lasts about five years.
- The proteinuric or albuminuric stage is characterized by the excretion of protein in the urine from the body, an increase in the size of the kidneys as a result of the development of hyperemia and lymphostasis, deposition of amyloid on the walls of blood vessels. During this period, sclerosis and lymphostasis often develops. This stage takes about twelve years.
- Edematous or nephrotic stage is caused by the appearance of a nephrotic syndrome with symptoms such as proteinuria, hypercholesterolemia and hypoproteinemia. Also in this case, the development of swelling, hypertension, splenomegaly is observed. Internal organs increase in size, intestines are affected. This stage takes about seven years.
- The terminal or azotemic stage is characterized by scarring and deformation of the kidneys, a decrease in their size, the development of renal failure and hypotension.
It is very important to identify kidney amyloidosis at an early stage of development, when the pathological process can be stopped. In the later stages of the disease, it is irreversible, recovery is possible in very rare cases. Renal failure can rapidly develop and lead to death at any of the above stages of the disease. Such a rapid progression of amyloidosis is caused by an exacerbation of the underlying disease, infection or renal vein thrombosis.
Diagnostic examination. What it is?
Renal amyloidosis, the symptoms and treatment of which are discussed in this article, is very difficult to diagnose at an early stage of development, since in this case the symptoms do not appear. The doctor in this case prescribes laboratory tests of blood and urine.
According to the results of the analysis of urine, it shows the presence of proteinuria, which tends to increase, as well as leukocyturia, microhematuria and cylindruria. The results of a blood test indicate an increase in ESR, anemia, leukocytosis, an increase in the level of sodium and calcium, cholesterol, and liver enzymes.
Also feces are taken for research. According to the results of the analysis, feces contain muscle fibers, a large amount of starch, as well as fats.
Typically, diagnosis of renal amyloidosis includes an electrocardiogram, which often detects arrhythmias and impaired signal conductivity, cardiomyopathy, and diastolic dysfunction. Ultrasound of the abdominal cavity and kidneys is often performed, which show an increase in the size of the liver and spleen, as well as kidneys, and radiography.
An effective diagnostic method is a kidney biopsy and further histological examination of the biopsy. When examining a biopsy specimen, it is stained, and the macropreparation "Large motley chain" is required for renal amyloidosis. In this case, the study shows the deformation of the kidneys, which have a wide cortical layer, large sizes. This method makes it possible to make an accurate diagnosis, to identify the stage of the disease.
It also involves the use of kidney amyloidosis micropreparation No. 128, which makes it possible to detect vascular congestion, glomerular infiltration with leukocytes, and endothelial cell proliferation.
When studying biological material, a laboratory technician can detect signs of other kidney diseases. A highly sensitive diagnostic test is RSK using an amyloid antigen.
Disease therapy
Renal amyloidosis treatment involves primarily the underlying disease. With the cure of the primary pathology, the symptoms of amyloidosis disappear for a long time. Patients are prescribed a diet in which it is necessary to eat raw liver for two years, limit the intake of salt and protein, increase the intake of carbohydrates and vitamins, potassium salts. Also, doctors recommend during this period to undergo treatment in a sanatorium, often to be in the fresh air, take vitamin complexes.
Renal amyloidosis clinical recommendations include the following:
- maintaining a healthy lifestyle, avoiding stressful situations;
- Exclusion of physical activity;
- getting rid of addictions;
- dieting.
Drug Therapy: Description
Renal amyloidosis also involves medication. The doctor prescribes antihistamines, for example, Suprastin or Diphenhydramine, antibacterial and anti-inflammatory drugs that have an immunosuppressive effect. To relieve puffiness, diuretics, antihypertensive drugs are prescribed, glucose in the form of droppers is prescribed to increase hemoglobin in the blood. Some doctors recommend treating kidney disease (amyloidosis) with corticosteroids and cytostatics. Sometimes the doctor may prescribe a course of chemotherapy or the use of the potent drug "Colchicine." But with prolonged use of these drugs, side effects develop in the form of dispersion, allergies, hematuria and leukopenia.
To stop the rapid progression of the disease can drugs from the group of aminocholines, for example, "Chloroquine" or "Delagil". With the use of these medicines for several years, provided that the pathology is diagnosed at an early stage of development, it is possible to significantly slow down the development of amyloidosis.
Surgical intervention
In severe cases, lifelong renal dialysis or transplantation of a donor organ is prescribed. A kidney transplant gives positive results when the cause of amyloidosis has been eliminated. Survival after such surgery is low, this is due to the development of amyloidosis of the heart and numerous complications. Relapses of the disease in the transplant also often develop, which can cause its loss. It is also possible to excise the affected area of the kidney.
With timely treatment of the disease, it is possible to reduce the pathological effect of amyloidosis on the internal organs, as well as eliminate unpleasant symptoms.
Forecast
Renal amyloidosis, the treatment of which at an early stage requires the elimination of the main disease, may have favorable prognoses. With the development of thrombosis, secondary infections, hemorrhages, the prognosis will be unfavorable, especially in older people. With the formation of heart and kidney failure, a person can live no more than a year. The formation of prolonged remissions is possible with timely therapy at an early stage of the disease, which should be effective.
Typically, the life expectancy of patients with this disease is from one to three years from the time of diagnosis. But in medical practice, cases were recorded when patients lived for more than ten years. The onset of death depends on the course of the underlying pathology. Very often, people die due to complications of the primary disease that caused the development of amyloidosis. Also, the cause of death is renal failure, which develops due to the rapid progression of pathology, the attachment of secondary infections that affect the intestines.
Prevention
With the aim of prevention, doctors recommend timely treatment of chronic diseases that can trigger the appearance of amyloidosis. Patients with this disease should be constantly monitored. While maintaining the functionality of the kidneys, dispensary observation is carried out twice a year with the obligatory diagnostic measures. In the early stages of the disease, doctors recommend visiting sanatoriums in the absence of serious pathologies in the body.
Amyloidosis of the kidneys is a serious disease that poses a danger to human life. Therefore, it is important to detect it in a timely manner in order to undergo effective therapy and prolong life. Today, many people die as a result of the development of renal failure. The number of cases of this disease grows annually. The disease affects people of various ages, including newborns. Often the disease is inherited through several generations, therefore it is important to observe the prevention of pathology to people who are at risk.