One of the most common forms of amyloidosis is renal amyloidosis. In most cases, the disease develops as a complication of such severe pathologies as tuberculosis, syphilis, osteomyelitis. Organ damage is a consequence of the underlying disease, which is recommended to be detected and treated in a timely manner. Pathology appears as a result of an increase in amyloid (a complex protein) in the blood, which settles on the walls of blood vessels and in the kidneys, as a result of which the functionality of the organ is disrupted. This disease occurs against the background of nephrotic syndrome and passes into the development of chronic renal failure. In severe cases, a donor kidney transplant may be required.
Problem description
Kidney amyloidosis is a complication of systemic amyloidosis, which is characterized by the accumulation of a glycoprotein substance, amyloid, in the intercellular space of the kidneys, which leads to disruption of the organ.
Amyloid is a product of the synthesis of pathological proteins as a result of dysproteinemia, which occurs due to a chronic disease. It is associated with autoimmune reactions. As a result of the development of a chronic disease, the decay products of toxins, leukocytes and tissues accumulate, which leads to the formation of antibodies, which, when reacted with antigens, form amyloids. These amyloids are deposited at sites where antibodies accumulate.
Secondary amyloidosis of the kidneys is of great importance in medical practice, as in most cases it leads to the formation of kidney 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 the developed countries of Europe, the disease occurs in 0.7% of cases, and in Asia - up to 0.2%. This is due to the difference in the diet of people. In the latter case, people often eat foods that contain small amounts 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 cases have been observed in children, including newborns.
Recently, the frequency of diagnosing pathology is growing. Especially often the disease is accompanied by pathology of the lungs, arthritis, ulcerative colitis, cancerous tumors. Often, amyloidosis develops in those people who have had tuberculosis or purulent infections. Amyloidosis is also often diagnosed in drug addicts who use narcotic drugs.substances in the form of injections, which often provokes the development of suppuration.
Disease types
In medicine, it is customary to distinguish the following types of pathology:
- Primary disease that appears as a result of a violation of the adhesion of immunoglobulins that 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 amyloidosis of the kidneys is formed due to chronic diseases: osteomyelitis, colitis, arthritis, sclerosis, tuberculosis or spondyloarthritis. 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.
Disease forms
In medicine, it is customary to distinguish several forms of the disease:
- Idiopathic form, in which the causes and mechanisms of the development of the disease are unknown.
- Family form, which is characterized 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. Usually 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.
- senilethe form is characterized by a disorder of protein metabolism.
- The local form has unexplained causes of development. It is usually associated with tumors of the endocrine system, diabetes and Alzheimer's syndrome.
- The tumor-like form also develops for unknown reasons. Doctors tend to argue that amyloidosis of this form develops due to certain types of neoplasms.
Causes of amyloidosis
Renal amyloidosis cause of appearance in most cases is unknown. Many diseases can provoke the appearance of complications in the form of amyloidosis, but it can also act as an independent disease.
In the primary pathology of the cause, it is not possible to establish it. Sometimes the disease is formed with multiple myeloma. In this case, in addition to the kidneys, the pathological process includes the tongue, skin, heart, lungs, liver, intestines and spleen.
Secondary amyloidosis of the kidneys is formed due to chronic infectious diseases and abnormal conditions a few 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, lymph nodes. Senile amyloidosis is a sign of aging and occurs in 80% of people over eighty.
The risk group includes people who have plasma cell disease, chronic inflammatory diseases, ankylosing spondylitis, rheumatoid arthritis, and Mediterranean fever.
Symptoms and signs of illness
Kidney 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 impairment of the affected organ, as well as the disease that caused 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, disorders of the cardiovascular system, and diarrhea appear. This disease provokes an increase in internal organs and the development of swelling. At a late stage in the development of the disease, hypertension, anuria and thrombosis of the renal veins are observed.
In medicine, the following symptoms of amyloidosis are recorded:
- impaired kidney function, development of kidney failure;
- disorder of the digestive tract;
- damage to the nervous system;
- development of arrhythmia and heart failure.
With amyloidosis, edema always develops. In some cases, renal vein thrombosis appears, pain syndrome, arrhythmia, dizziness, and shortness of breath develop.
Stages of kidney amyloidosis
During the course of the disease, several stages are distinguished, each of which is characterized by 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 a slightproteinuria. This period lasts about five years.
- Proteinuric or albuminuric stage is characterized by the excretion of protein from the body with urine, an increase in the size of the kidneys as a result of the development of hyperemia and lymphostasis, the deposition of amyloid on the walls of blood vessels. During this period, sclerosis and lymphostasis often develop. This stage takes about twelve years.
- Edematous or nephrotic stage is caused by the appearance of nephrotic syndrome with symptoms such as proteinuria, hypercholesterolemia and hypoproteinemia. Also in this case, the development of edema, hypertension, splenomegaly is observed. The internal organs increase in size, the intestines are affected. This stage takes about seven years.
- 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 detect amyloidosis of the kidneys at an early stage of development, when it is possible to stop the pathological process. In the later stages of the disease, it is irreversible, recovery is possible in very rare cases. Kidney failure can progress rapidly and be fatal at any of the above stages of the disease. Such a rapid progression of amyloidosis is caused by an exacerbation of the underlying disease, the addition of an infection or thrombosis of the renal veins.
Diagnostic examination. What is it?
Kidney amyloidosis, the symptoms and treatment of which are discussed in this article, is very difficult to diagnose at an early stagedevelopment, 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 can be seen that the presence of proteinuria, which tends to increase, as well as leukocyturia, microhematuria and cylindruria, is noted in it. The results of a blood test indicate an increase in ESR, anemia, leukocytosis, an increase in the level of sodium and calcium, cholesterol, liver enzymes.
Also, feces are taken for research. The analysis shows that the feces contain muscle fibers, a large amount of starch, as well as fat.
Typically, the diagnosis of renal amyloidosis involves an electrocardiogram, which often shows arrhythmia and signal conduction disturbances, cardiomyopathy, and diastolic dysfunction. Often, ultrasound of the abdominal cavity and kidneys is performed, which show an increase in the size of the liver and spleen, as well as kidneys, x-rays.
An effective diagnostic method is kidney biopsy and further histological examination of the biopsy. When examining a biopsy specimen, it is stained, while amyloidosis of the kidney of the “Big motley chain” macropreparation is required. 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.
Also involves the use of amyloidosis of the kidney micropreparation No. 128, which makes it possible to detect vascular plethora, infiltration of glomeruli by leukocytes andproliferation of endothelial cells.
When examining biological material, a laboratory assistant can detect signs of other kidney diseases. A highly sensitive diagnostic test is RSK using an amyloid antigen.
Therapy of disease
Renal amyloidosis treatment involves primarily the underlying disease. When the primary pathology is cured, the symptoms of amyloidosis disappear for a long time. Patients are prescribed a diet in which it is necessary to consume raw liver for two years, limit the intake of s alt and proteins, increase the intake of carbohydrates and vitamins, potassium s alts. Also, doctors recommend during this period to undergo treatment in a sanatorium, often go outdoors, take vitamin complexes.
Renal amyloidosis clinical guidelines suggest the following:
- maintaining a he althy lifestyle, avoiding stressful situations;
- exclusion of physical activity;
- getting rid of bad habits;
- dieting.
Drug Therapy Description
Renal amyloidosis treatment also involves medication. The doctor prescribes antihistamines, for example, Suprastin or Dimedrol, antibacterial and anti-inflammatory drugs that have an immunosuppressive effect. To relieve swelling, 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)corticosteroids and cytostatics. Sometimes the doctor may prescribe a course of chemotherapy or the use of the potent drug Colchicine. But with long-term use of these drugs, side effects develop in the form of dispersion, allergies, hematuria and leukopenia.
Stop the rapid progression of the disease can drugs from the group of aminocholines, such as Chloroquine or Delagil. With the use of these drugs for several years, provided that the pathology is diagnosed at an early stage of development, it is possible to greatly slow down the development of amyloidosis.
Surgery
In severe cases, lifelong kidney dialysis or a donor organ transplant is prescribed. Kidney transplantation gives positive results when the cause of amyloidosis is eliminated. Survival after such surgical intervention is low, this is due to the development of cardiac amyloidosis and numerous complications. Also, relapses of the disease in the graft often develop, which can cause its loss. It is also possible to perform excision of the affected area of the kidney.
With timely treatment of the disease, it is possible to reduce the pathological effect of amyloidosis on internal organs, as well as eliminate unpleasant symptoms.
Forecast
Renal amyloidosis, whose early treatment requires elimination of the underlying disease, may have a favorable prognosis. With the development of thrombosis, secondary infections, hemorrhages, the prognosis will be unfavorable, especiallyThis is true for older people. With the formation of heart and kidney failure, a person can live no more than a year. The formation of long-term remissions is possible with timely therapy at an early stage of the disease, which should be effective.
Typically, patients with this disease have a life expectancy of one to three years from the time of diagnosis. But in medical practice, cases have been 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 kidney failure, which develops due to the rapid progression of the pathology, the addition of secondary infections that affect the intestines.
Prevention
For the purpose of prevention, doctors recommend timely treatment of chronic diseases that can provoke the appearance of amyloidosis. Patients with this disease should be under constant supervision. While maintaining the functionality of the kidneys, dispensary observation is carried out twice a year with mandatory diagnostic measures. In the early stages of the disease, doctors recommend visiting sanatoriums in the absence of serious pathologies in the body.
Kidney amyloidosis is a serious disease that poses a threat to human life. Therefore, it is important to detect it in time in order to undergo effective therapy andprolong life. Today, many people die as a result of developing kidney failure. The number of people with this disease is growing every year. The disease affects people of all ages, including newborns. Often the disease is inherited through several generations, so it is important to observe the prevention of pathology for people who are at risk.
