Primary biliary cirrhosis is a destructive and inflammatory liver disease characterized by a chronic course. It is accompanied by damage to the intrahepatic ducts. As such cirrhosis progresses, ductopenia (disappearing bile duct syndrome) and persistent cholestasis (decreased bile flow into the duodenum due to disturbances in its production, excretion and/or excretion) may develop. In the terminal stage of the disease, liver failure is usually observed.
Symptoms
At an early stage, primary biliary cirrhosis may be asymptomatic or masked as manifestations of other diseases. As liver damage progresses, the following symptoms may occur.
Clinical signs
- Skin itching
. At first it occurs from time to time, then it becomes constant, often intensifying at night, which significantly reduces the quality of life. Patients may experience scratching on the skin of the arms, thighs, and back. Itching may be the only sign of primary biliary cirrhosis until cholestatic jaundice appears. Sometimes the symptom persists for many years. - Jaundice
. Typically occurs 6–24 months after the onset of itchy skin. There is yellowing of the skin, enlargement of the liver and spleen, acholic (light) stools, darkening of the urine (with a positive reaction to bilirubin). - Other symptoms
. Primary biliary cirrhosis may be accompanied by pain in the right hypochondrium, muscles and joints, fever, and the appearance of skin xanthelasmas (cholesterol deposits) in the palms, elbows, eyelids, and buttocks.
Laboratory signs
. With this cirrhosis, characteristic biochemical abnormalities are observed: increased bilirubin, alkaline phosphatase, ALT and AST transaminases, gamma-glutamyl transpeptidase (GGTP). In addition, lipid profiles change in patients. In many cases, when studying humoral immunity, an increased level of immunoglobulin class M is revealed. An increase in ESR and moderate anemia are also often detected.
Classification of the disease
Biliary cirrhosis of the liver is assessed using the Child-Pugh scale, which implies an assessment of the functioning of the organ and:
- the presence of ascites and encephalopathy (one of the most common complications of liver diseases);
- bilirubin level;
- albumin concentration;
- prothrombin index, which indicates the presence of blood clotting disorders.
The difference between a healthy liver and a liver affected by cirrhosis
After studying all these indicators, points are given (from 0 to 10), then they are summed up and the class of biliary cirrhosis is determined. In total, there are 3 classes of this disease:
- A – it is characterized by minimal scores, the average life expectancy after surgery is 20 years;
- B – the number of points is average, after surgical interventions, life expectancy is on average 10 years, strip operations end in death in 30% of cases;
- C – high score, surgical interventions are rarely performed, abdominal operations end in death in 80% of cases.
There is also another classification of biliary cirrhosis of the liver - METAVIR, which is used to determine the degree of damage to the organ by fibrous tissue. This assessment is carried out through a biopsy. Gradation of degree - from 0 to 4.
Complications
Intrahepatic cholestasis in primary biliary cirrhosis can cause a lack of bile in the intestine, which can be accompanied by steatorrhea (an increase in the amount of fatty components in the stool), the development of a deficiency of fat-soluble vitamins, impaired bone mineralization and, as a consequence, the occurrence of osteoporosis. As liver disease progresses, patients gradually lose weight and usually develop symptoms such as blurred vision, bone pain, bleeding gums, and possible pathological fractures.
general description
Biliary cirrhosis is
a chronic autoimmune disease that occurs as a result of a violation of the outflow of bile through the intrahepatic and biliary tract (cholestasis) and is characterized by the replacement of parenchymal liver tissue with connective tissue (fibrosis). The prognosis of the disease itself depends on diagnosis and in undiagnosed cases leads to deterioration of development: progressive destruction of the parenchyma with the formation of foci of fibrosis, which results in liver cirrhosis and liver failure.
According to statistics, in economically developed countries, biliary cirrhosis is diagnosed in people aged 30 to 55 years, more often in men. The ratio of incidence among men to women is approximately 3 to 1.
Treatment
Pathogenetic therapy
. This treatment is prescribed to stop the progression of primary biliary cirrhosis, improve the patient’s quality of life and increase its duration. As part of pathogenetic therapy, ursodeoxycholic acid preparations, which have anticholestatic, cytoprotective, immunomodulatory and hypocholesterolemic effects, can be used. The drug Phosphogliv®1 can be used as part of complex therapy for cirrhosis.
Use of corticosteroids
. To achieve regression of the clinical symptoms of the disease, corticosteroid hormones (prednisolone or its analogues) can be prescribed. The use of such drugs can improve biochemical and histological parameters and increase life expectancy.
Symptomatic therapy
. To reduce skin itching, sorbents and agents are usually prescribed that can reduce the flow of bile acids into the enterohepatic circulation. For steatorrhea and bile deficiency in the intestines, enzyme preparations with high levels of lipase can be used. If the patient is experiencing severe bone pain, parenteral administration of calcium supplements may be indicated. They are also used to prevent and treat osteoporosis.
Surgery
. Liver transplantation may be prescribed if the level of serum bilirubin exceeds 150 µmol/l, there are signs of decompensation of the process (hepatic encephalopathy, repeated bleeding from the esophageal veins, etc.). Before the development of cirrhosis, surgical treatment can be used in connection with severe osteoporosis, intolerable skin itching, and a significant decrease in quality of life.
1 According to the instructions for medical use of the drug.
Causes:
Due to their occurrence, there are 2 types of biliary cirrhosis:
- Primary biliary cirrhosis of the liver
- Secondary biliary cirrhosis of the liver
Primary biliary cirrhosis - the mechanism of occurrence is that autoimmune inflammation occurs in the liver tissue itself. Antibodies are produced against liver cells (hepatocytes) and they are perceived by the human body as foreign. The process itself is joined by a protective system, in the form of lymphocytes, macrophages, mast cells, which produce biologically active substances and antibodies. All of them together destroy hepatocytes, cause disturbances in blood supply, metabolism and bile stagnation, leading to general destruction of the architectonics (structure) of the liver.
- Genetic predisposition
- People suffering from autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, thyrotoxicosis, scleroderma, polyarteritis nodosa, sarcoidosis
- It is known from scientific sources that about 15% of cases of the disease are caused by an infectious substrate such as the herpes virus, rubella, Epstein-Barr
Secondary biliary cirrhosis of the liver occurs due to blockage or narrowing of the lumen of the intrahepatic bile ducts.
- Developmental anomalies (congenital or acquired) of the bile ducts and gallbladder
- Cholelithiasis
- Narrowing or blockage of the biliary tract after surgery on the abdominal organs, benign tumors
- External compression of the biliary tract by an inflamed pancreas or tumor
Toxic cirrhosis of the liver
The disease occurs against the background of intoxication. This could be minor poisoning, systematic use of toxic substances. For example, antibiotics, non-steroidal anti-inflammatory drugs, taking anabolic steroids and others. Working with chemicals can also lead to the development of liver pathology. Some herbs can affect the body when brewed almost on par with mercury and cadmium.
With gradual poisoning, progressive destruction occurs. However, symptoms can appear already at the most life-threatening stages; they manifest themselves acutely, with a whole set of unpleasant signs.
- increased fatigue, loss of appetite, sudden weight loss;
- the temperature rises, hypothermia may occur;
- yellowness of the skin and eyes;
- the level of hemoglobin in the blood drops;
- the condition of the skin changes: itching appears, a vascular network forms;
- pain on the right (if there is no pain with other symptoms, this indicates a worsening situation);
- an increase in the size of the abdomen, vomiting blood, blood from the nose (internal bleeding).
The toxic form can lead to mental disorders and impaired consciousness.
Diagnostics
Diagnosis of liver cirrhosis begins with a clinical examination, which includes collecting complaints and examination.
During examination, the doctor may detect external signs of CP1:
- Redness on the palms and soles (palmaral erythema)
- Spider veins
- Reduction of hair in the armpits
- White nails
- Men with cirrhosis of the liver may experience gynecomastia, an enlargement of the mammary glands. The reasons for this are considered to be a reduced level of testosterone and an increased level of estradiol (the female sex hormone) in the blood due to various mechanisms: increased processes of aromatization of testosterone into estradiol, as well as increased production of globulin that binds sex steroids, and thus a decrease in the level of free testosterone.6 .7
Later, edema may appear, especially in the lower extremities, jaundice, and the development of ascites - accumulation of free fluid in the abdominal cavity.
When palpating the abdomen, an enlarged liver is detected; it is often possible to palpate the nodal surface of the organ, the pointed edge. However, in the final stage of the disease, the size of the liver may decrease, and the spleen may increase.1 To confirm the diagnosis and establish the stage of the disease, laboratory and instrumental studies are carried out, and calculated indices are also used.
Laboratory methods
- Standard biochemical tests that determine the level of bilirubin, total protein, prothrombin index, serum iron, ferritin and enzyme activity (AST, ALT, alkaline phosphatase). These tests are nonspecific, their deviations can be observed in other diseases, but they help assess the degree of liver dysfunction.
- General clinical blood tests can reveal the presence of anemia, a decrease in the number of leukocytes, platelets, which can also be observed with cirrhosis.
- Tests for hepatitis viruses and other infectious agents may be ordered to determine the potential cause of the disease.
Calculation indices:
The use of test panels, such as FibroTest, FibroIndex, Hepascore, can be used to assess the stage of fibrosis. For example, the use of the Fibrotest score avoided biopsy in 50% of patients.2
To assess the severity of cirrhosis and predict survival, the Child-Pugh classification is most often used.2 The scale, which takes into account the results of laboratory tests and clinical manifestations, allows one to assess the degree of functional impairment and determine the stage of the disease. The higher the score, the worse the prognosis.2
Instrumental studies
In addition to needle biopsy, which is recognized as the “gold standard” for diagnosing fibrosis and cirrhosis, liver imaging methods play an important role in diagnosis. These include:
- Ultrasound of the liver, abdominal organs and retroperitoneal space. The most accessible and fairly informative method. Allows you to evaluate the anatomical and structural features, shape, size, vascular pattern. CP can be judged by an increase in the size of the liver and by its heterogeneous structure and uneven tuberous contour. Also, with liver cirrhosis, ultrasound may show signs of portal hypertension: ascites, dilation of portal veins, enlarged spleen.1
- CT, MRI (computer and magnetic resonance imaging of the liver) - by performing a large number of thin sections, it provides comprehensive information about the structure of the organ, the presence of fibrous nodes, false lobules, additional vessels, etc.
- Ultrasound elastometry (FibroScan device) can serve as an alternative to liver tissue biopsy, as it makes it possible to assess the density of the organ, which increases as the percentage of connective tissue increases.2
Only a comprehensive diagnosis can give a complete picture of the disease, so a combination of various methods is used, which sometimes have to be repeated several times.
Alcoholic cirrhosis of the liver
The disease is triggered by regular alcohol consumption. The usual “100 grams” every day after work may well lead to the development of the disease. The body suffers especially if the patient eats abnormally. A small amount of food or accompanying consumption of harmful food significantly increases the risks.
Liver cirrhosis: stages and symptoms in alcoholic form
At the initial stage, the pathology does not make itself felt and develops hidden. However, hardware studies reveal increased sizes of the organ.
This type of disease is characterized by reproductive dysfunction: women experience spontaneous abortions, men experience impotence and testicular atrophy. The same disorders include hormonal imbalances in the body.
How does the alcoholic form of the disease manifest:
- bowel dysfunction;
- loss of appetite;
- regular attacks of nausea and vomiting;
- loss of sensation in the limbs;
- muscle tissue becomes thinner.
Among the subtle symptoms are protein and vitamin deficiency in the body.
Diet
Diet plays an important role in the treatment of biliary cirrhosis. With the development of this disease, the functioning of not only the liver, but also other internal organs fails. And in order to reduce the burden on them and prevent the development of complications, all patients, without exception, need to follow certain rules in their diet. Depending on the severity of the disease and its accompanying complications, patients are assigned to treatment table No. 5a or No. 10 (mainly used when ascites occurs).
Violation of nutritional rules in biliary cirrhosis of the liver shortens the patient’s life
The daily calorie intake should not exceed 2900 kcal. In this case, it is imperative to refuse:
- fatty and fried foods;
- alcoholic drinks;
- dairy products;
- honey;
- fatty fish and meat;
- seafood;
- nuts
The main part of the diet should be fresh vegetables and fruits. You need to eat food in small quantities, but often - about 5-6 times a day. The last meal should be 2-3 hours before bedtime.
Food should be consumed boiled. Some dishes can be steamed. It is not advisable to bake food in the oven even without using oil and spices. In this case, food should be eaten warm. If liver function is impaired, hot and cold foods and drinks should not be consumed. Fasting days are held once every 2 weeks, during which the patient must eat only fresh vegetables and fruits.
Vegetables and fruits provide the body with vitamins and minerals, maintaining its functionality at the proper level.
An important point is compliance with the drinking regime. This allows you to avoid the occurrence of edema and complications in the form of disorders of the urinary system. Patients with biliary cirrhosis are recommended to drink at least 2 liters of purified water per day (this volume does not include teas and liquid dishes).
Review of drugs
Drug therapy only stops pathological processes in the digestive gland and does not lead to recovery. With the help of hepatoprotectors, antibiotics, diuretics and other medications, it is possible to reduce the severity of symptoms and thereby alleviate the patient’s condition. Biliary cirrhosis can be treated with the following drugs:
- cytostatics (“Aminosol”, “Decapeptyl”) - antitumor medications that prevent the formation of malignant tumors;
- choleretic drugs (“Nicodin”, “Hologon”) - increase the concentration of acids in bile and stimulate its production in the digestive gland;
antioxidants (“Essentiale”, “Complivit Selenium”) - neutralize harmful substances and free radicals in the body;- hepatoprotectors (“Heptral”, “Karsil”) - protect hepatocytes from the negative effects of toxins, antibodies and other substances;
- diuretics (“Spironol”, “Aldactone”) - promote the excretion of salts and toxic substances in the urine;
- anti-inflammatory drugs (“Essliver Forte”, “Kuprenil”) - reduce inflammation in the parenchyma, thereby slowing down the process of death of hepatocytes.
Irrational use of diuretics can lead to impaired renal function and the development of encephalopathy.
