Renal Amyloidosis: Causes, Symptoms and Treatment

Renal Amyloidosis

Renal amyloidosis is rare, but this pathology should not be ignored. As statistics show, the disease affects 1 person out of 50-60,000 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 have been 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.

Content:

Amyloidosis – What is It?

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

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 contains no 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 response to it. There is no struggle. While an excess of amyloid has a negative impact on health.

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

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 such as urea, uric acid and creatinine remain in it.

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 the cells of the immune system to produce abnormal protein compounds that the body transforms into amyloid.

Secondary Amyloidosis. This type of disease is caused by another disorder in the body. Chronic pathology leads to a malfunction of the immune cells.

Senile Amyloidosis. As we age, the body ceases to function fully. This applies to all systems, including immunity. The number of leukocytes in older people remains at the same level as in young people, but the metabolism in them undergoes certain changes. Therefore, the body begins to produce pathological compounds, which include amyloid.

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

  • Individuals with a family history of amyloidosis or other renal pathologies of unclear etiology.
  • People Over 65 -Persons with rheumatoid arthritis, ankylosing spondylitis, SLE, cancer.

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

Amyloidosis Symptoms

The disease develops over time. Symptoms increase gradually. From the moment the first signs of a disorder appear until the onset of pronounced symptoms, it can take more than 10 or even 20 years. Despite such a slow progression of pathology, it is possible to identify it too late to prescribe an effective therapy to the patient.

Renal Amyloidosis Symptoms
Renal Amyloidosis Symptoms

The First Symptoms of Renal Amyloidosis

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

The loss of protein in the urine does not pose a threat to the body, since very little of it is released. 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 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 are at risk need to donate urine for analysis twice a year. To do this, it is enough to contact the polyclinic 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 humans. Therefore, with urine per day, he will lose about 3 gm of protein, with a normal loss of 0.13 gm/l. At the same time, edema begins to appear in patients. Their intensity varies, ranging from a slight swelling of the face and ending with the sweating of fluid into tissues and organs.

Other Symptoms of Amyloidosis:

Urination becomes more frequent, becomes profuse. The kidney filter is unable to perform its functions, so up to 10 liters of fluid are excreted in the urine per day. This disorder is called renal diabetes. Although the loss of fluid is significant, this does not allow 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 rise or fall. It is impossible to predict what kind of failure will happen in a particular patient. However, most often people suffer from low blood pressure.

The color of the urine is unusual. It turns reddish. This symptom indicates that the kidneys are being destroyed.

A set of extra pounds. Weight gains due to edema. In addition, impaired kidney function leads to a malfunction in fat metabolism. The patient’s level of cholesterol, triglycerides and lipoproteins rises. Fat begins to be deposited in tissues, the figure on the scales creeps up.

Doctors call this symptom complex amyloid-lipoid nephrosis. The duration of this stage is 6 years (average 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 enter 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, organs lead to the retention of toxins (uric acid, urea, bilirubin, creatinine, etc.).

Symptoms that come to the fore:

  • Great weakness.
  • Deterioration of attention.
  • Headaches.
  • 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 disruption in the work of other internal organs will also appear, since amyloid will also begin to accumulate in them.

Such violations include:

Skin Lesions. Semi-transparent nodules appear on the face, neck, armpits and in the popliteal fossa. The skin under the eyes will be soaked in blood. It becomes dry in the wrist area.

Heart Failure. The patient has shortness of breath, which occurs after exercise. He is also worried about chest pains localized on the left side.

Damage to the Digestive Tract. The tongue increases in size, teeth imprints are visible on it. A few hours after eating, pains appear in the right side of the abdomen. The feces become black, shiny and extremely offensive. This symptom indicates stomach or intestinal bleeding. The chair may be absent for 3 days or more.

Damage to the Joints. In the morning, the patient experiences stiffness in the limbs. It becomes less intense after movement.

Damage to the Nervous System. Feet and hands lose sensitivity, the skin on them becomes less elastic, begins to peel off.

Even one of the listed symptoms is a reason to suspect amyloidosis.

Diagnostics

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

Analysis NameCharacteristic Changes
HOW (clinical blood test)– ESR accelerates to 15 mm/hour.
– Leukocytes reach 9 x 109 cells/l.
LHC (biochemical blood test)– The level of C-reactive protein reaches 5 mg/l.
– The creatinine level rises above 110 mmol/l.
– The urea level increases to 7.6 mmol/l.
– The uric acid level rises to 400 mmol/l.
– The total blood protein level is reduced to 67 gm/l.
KAM (clinical urinalysis)– The protein level in urine rises to 0.14 gm/l.
– Hyaline casts, glucose, erythrocytes and leukocytes are found in urine.

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

Puncture of The Subcutaneous Tissue. 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 makes it possible to diagnose amyloidosis.

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

In addition, the patient is referred for renal ultrasound, 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 Renal Amyloidosis

Therapy of renal amyloidosis is difficult, since the disease is difficult to correct. It is impossible to remove amyloid from organs by 100% using drugs or physiotherapy. Provided treatment is started early, the rate of progression of the pathology can be reduced and serious kidney damage prevented.

Treatment of Renal Amyloidosis
Treatment of Renal Amyloidosis

Compliance with a diet. Diet food is the most important condition for the therapy 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 in the menu such substances as:

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

Casein. This substance is found in dairy products. It is also found in formula for athlete’s nutrition.

Beef and Veal. The menu can include cereals, vegetables, lamb, chicken. Pastry products are excluded, which is especially important for overweight people.

Taking Medications

Drug NameHow does it work?Side Effects
UnithiolThe drug binds proteins from which amyloid is formed. 
This allows you to slow down the progression of pathology.
The person may feel dizzy, have an increased heart rate, or feel sick.
DimexideThe course intake of this drug can significantly improve the patient’s well-being, but due to what this happens until now it is unknown.Patients may experience allergies, digestive disorders, vomiting.
Delagil or PlaquenilThese drugs block the production of certain enzymes, without which the production of amyloid is slowed down. 
These funds are highly effective in early detection of the disease.
Dyspeptic symptoms, emotional instability, decreased immunity, decreased visual acuity.

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 used only in extreme cases, when the patient’s life is in real danger. In addition, finding a suitable donor in Russia is rather problematic. Therefore, such an operation is rare.

Forecast

If treatment is started on time, then 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 happens with the rapid progression of the pathology, or with its late detection.

1. 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 exactly Colchicine, then you should heed his advice.

2. What are the risks of inherited disease?

Such risks exist. They increase if several family members have a history of amyloidosis. To reduce them, before conception, you need to minimize the intensity of the pathological process.

3. Are there measures to prevent the development of amyloidosis?

No such measures have been developed.

4. Can the disease recur after kidney transplant?

Such a probability exists, and it is quite high. However, it takes about 15 years from the onset of the disease to the onset of the first symptoms. If the treatment is started on time, then this period will increase by 2-3 times. Therefore, repeated kidney damage simply does not have time to develop again.