Clinic
Clinical signs of the disease appear gradually. Moreover, in half of the patients, primary biliary cirrhosis is asymptomatic for a long time.
The most characteristic manifestations of primary biliary cirrhosis are: increased fatigue, cholestasis (which leads to impaired fat absorption, multivitamin deficiency, osteoporosis), as well as hepatocellular dysfunction.
Clinical symptoms in the early stages of the disease
The early signs of primary biliary cirrhosis (occur in more than 50% of cases) are:
Itchy skin.
Itchy skin occurs in 55% of patients and almost always precedes (by 6-18 months) the appearance of jaundice . In 10% of cases, severe itching is observed. In some patients, itching and jaundice occur simultaneously.
Fatigue (in 65% of patients).
In some cases, this symptom leads to a decrease in patients' work capacity and is often accompanied by depressive reactions and obsessive-compulsive syndrome. Patients are concerned about increased drowsiness, especially in the daytime.
Dry eyes.
Less often in the initial period of the disease there is a feeling of discomfort in the upper right quadrant of the abdominal cavity (in 8-17% of patients).
In the later stages of the disease (usually several years after the manifestation of primary biliary cirrhosis), signs of decompensation of liver function are detected:
Ascites.
Spider veins.
Hepatic failure and encephalopathy .
In 10% of patients around the eyes and on extensor surfaces of the joints, xanthelasma and xanthomas are formed.
Xanthelasma (yellow, slightly rising plaques around the eyelids).
In 25% of cases, hyperpigmentation of the skin is detected.
In 10% of cases, jaundice occurs .
ellowness sclera.
Jaundice - the skin becomes yellow.
In 50-75% of patients, Sjogren's syndrome develops, the main manifestations of which are: xerophthalmia (dry eyes), xerostomia (dry mouth).
Very rarely, a Kaiser-Fleischer ring can be found in patients: deposition of a yellow-brown pigment along the periphery of the cornea (in the descemet casing).
In the genesis of the disease, a certain role is given to genetic factors. Thus, the risk of a disease among relatives of the patient’s first line of kinship is many times higher than the same indicator among people whose families do not have patients with primary biliary cirrhosis. Therefore, when collecting an anamnesis, it is important to consider the state of health of the patient’s closest relatives.
It is necessary to clarify when the first complaints of the patient appeared, and how the disease subsequently developed.
Physical research
Laboratory diagnostic methods
Instrumental diagnostic methods
Additional examination of patients with primary biliary cirrhosis
Diagnostic algorithm
The diagnosis of the disease is based on the clinical symptoms of the disease, the results of laboratory tests, imaging methods (ultrasound, CT, MRI) and histological examination of liver biopsy specimens.
So, a suspicion of primary biliary cirrhosis should arise if patients (usually women 40-60 years old) complain of skin itching, increased fatigue, a feeling of discomfort in the upper right quadrant of the abdomen). The diagnosis is confirmed when an alkaline phosphatase and gamma-glutamyl transpeptidase ( 3-4 times higher values of alanine aminotransferase and aspartate aminotransferase are detected slightly in the biochemical analysis of blood ).
The bilirubin content in the early stage of the disease remains within the normal range and increases as the disease progresses.
A marker of the disease is the detection of antimitochondrial antibodies (AMA) in serum. In some patients with clinical, histological and biochemical signs of primary biliary cirrhosis, AMA is not detected. In these cases, patients are diagnosed with autoimmune cholangitis.
Imaging diagnostic methods are performed to rule out the possibility of biliary obstruction.
A liver biopsy is performed to establish or confirm a diagnosis, clarify the stage of the disease and evaluate the prognosis.
The presence of cholelithiasis can be suspected in the presence of paroxysmal pain in the right hypochondrium, especially accompanied by jaundice.
Diagnostic Objectives
Determination of the presence of calculi in the biliary tract and their relationship with clinical manifestations.
Characterization of bile calculi - localization, chemical composition, dimension and quantity.
Identification of complications.
Methods for the diagnosis of gallstone disease
In the diagnosis of gallstone disease, along with the characteristic clinical picture, an important role is played by imaging and laboratory diagnostic methods.
Medical history
When collecting an anamnesis, the limitation of the appearance of such symptoms (paroxysmal pain in the right hypochondrium or epigastrium), their dynamics and frequency of occurrence should be clarified. It is necessary to find out whether the examination was carried out (to obtain data from previous studies for comparison) and the treatment of this disease earlier.
Physical examination
An important role is played by an objective examination of the patient during an attack of pain. In this case, symptoms of muscle protection, increased pain during palpation and percussion of the abdomen in the right hypochondrium, as well as increased pain during palpation of the gallbladder at the inspiratory height - positive symptoms of Murphy and Kera can be determined.
Laboratory diagnostic methods
Laboratory indicators for uncomplicated cholelithiasis, as a rule, are not changed.
After an attack of biliary colic, in 40% of cases there is an increase in the activity of serum aminotransferases ( ALT and AST ), in 23-25% of alkaline phosphatase , γ-glutamyl transpeptidase , in a 45-50% increase in the content of bilirubin . A week after the attack, indicators usually return to normal.
If the course of the disease is complicated by the development of acute cholecystitis, then leukocytosis and an acceleration of ESR are noted .
Visual and instrumental diagnostic methods
The leading role in the diagnosis of gallstone disease is played by imaging research methods.
Ultrasound of the abdomen
Abdominal x-ray
Computed tomography (CT)
Biliary tract scintigraphy with 99mTc-substituted iminodiacetic acids (HIDA scintigraphy)
Endoscopic Pancreatocholangiography (ERCP)
Magnetic Resonance Pancreatocholangiography (MRPC)
Endoscopic Ultrasonography (EUS)
Cholecystography (oral and intravenous)
Treatment
Treatment goals
A decrease in the intensity of the clinical manifestations of the disease.
Preventing or slowing the progression of the disease.
Prevention and treatment of complications.
Treatment methods
Non-drug activities
It is important to change the lifestyle: stop drinking alcohol, taking hepatotoxic drugs. Activities related to nonspecific therapy are aimed at achieving changes in the diet and lifestyle of patients with cirrhosis. They also include restrictions on the intake of certain medications, compliance with a certain level of physical activity.
It must be remembered that patients with cirrhosis should be actively treated for concomitant infections; prophylactic prescription of antibiotics during any instrumental manipulations (treatment by a dentist, laparoscopy, catheterization) is indicated. At the slightest sign of decompensation, bed rest and inpatient treatment are recommended.
It is forbidden to conduct stress studies, balneological and physiotherapeutic procedures, insolations.
Patients with chronic liver diseases are recommended vaccination against hepatitis A, B, pneumococcal infection and influenza.
Nutrition for patients with cirrhosis.
Patients in the compensated stage of cirrhosis are prescribed a balanced rational diet. Alcohol should be discarded. Read more: Clinical nutrition for liver diseases ).
A low-protein diet is indicated for patients at high risk of developing hepatic encephalopathy. However, in such patients there is a likelihood of muscle weakness. With the development of signs of encephalopathy, protein nutrition should be reduced to 40.0 g per day with a uniform distribution throughout the day.
With ascites , a salt-free diet is indicated.
Physical activity.
After consulting a doctor, maintain a certain level of physical activity (for the prevention of osteoporosis). In the compensated stage, patients can perform work that is not related to the forced position of the body, prolonged walking or standing, or fluctuations in ambient temperature. Regular physical activity (walking, swimming) is recommended.
Patients with a more severe course of the disease can also carry out special complexes of physical exercises under the supervision of an instructor.
Drug restriction.
Patients with chronic liver disease and cirrhosis should avoid taking non-steroidal anti-inflammatory drugs ( indomethacin , ibuprofen ( Nurofen )), isoniazid , valproic acid ( Depakin , Konvuleks ), erythromycin , amoxicillin / clavulanate ( Amoxiclav , Augmentin , ketoconazole ( Nizoral ), chlorpromazine ( Aminazine ), ezetimibe ( Ezetrol ), aminoglycosides.
In patients with decompensated cirrhosis of the liver, non-steroidal anti-inflammatory drugs can lead to gastrointestinal bleeding, renal failure.
It is also recommended to limit the use of medicinal herbs and dietary supplements to food as much as possible.
For the treatment of primary biliary cirrhosis are used: immunosuppressants, corticosteroid hormones, drugs that enhance the excretion of copper, ursodeoxycholic acid.
Ursodeoxycholic Acid (UDCA).
Treatment of primary biliary cirrhosis with ursodeoxycholic acid ( Ursofalk , Ursosan ) should be started already at the first stage (stage of inflammation) or, at least, at the second stage (stage of progressive inflammation) of the disease.
UDCA has a positive effect on such prognostic factors of primary biliary cirrhosis as: the level of serum bilirubin, the severity of liver tissue necrosis, the progression of histological changes, ascites and edema, as well as the severity of skin itching and the intensity of general weakness.
Against the background of the use of ursodeoxycholic acid, indications for liver transplantation are less likely to occur, and the life expectancy of patients increases. At stages I and II of primary biliary cirrhosis, the life expectancy of patients is 20–25 years, therefore, the therapeutic effectiveness of UDCA in the late stages is markedly reduced.
With the use of UDCA at a dose of 10-15 mg / kg / day, the level of laboratory parameters characteristic of primary biliary cirrhosis - alkaline phosphatase, gamma-glutamyl transpeptidase, immunoglobulin M and aminotransferases significantly decreases (compared with the initial level).
In approximately 20% of patients, treatment with the drug may be ineffective.
Immunosuppressants.
Immunosuppressants inhibit immune responses and prevent disease progression.
Methotrexate (Methotrexate-Ebeve) improves the biochemical and histological parameters of the disease. It is administered orally at a dose of 15 mg per week.
Cyclosporin ( Sandimmun-Neoral ) is administered orally at a dose of 3 mg / kg / day (in 2 divided doses).
Anti-inflammatory therapy.
Nonsteroidal anti-inflammatory drugs and corticosteroid hormones can be used in low doses. Not prescribed during pregnancy and lactation.
Corticosteroid hormones can reduce the severity of clinical manifestations of primary biliary cirrhosis and improve biochemical and histological parameters of the disease. When prescribing, it is necessary to remember the possibility of developing osteoporosis during treatment.
Prednisoloneum ( Prednisoneumum table. ) Is appointed inside at a dose of 30 mg / day. Within 8 weeks, the dose is reduced to a maintenance dose of 10 mg / day.
Treatment of copper metabolism disorders.
In patients with primary biliary cirrhosis, copper metabolism is impaired. Therefore, it is necessary to use drugs that contribute to its removal from the body.
The drug of choice is D-penicillamine ( Cuprenyl ). It is a copper chelator. It is applied orally, on an empty stomach (one should not eat food 1.5 hours before and after ingestion). Adults are prescribed with a gradual increase in dose from 250 mg / day (up to 2 g per day). The dose is divided into 4 doses. The dose is increased by 250 mg per week.
Itchy skin treatment.
Itchy skin is often resistant to therapy and significantly impairs the quality of life of patients with primary biliary cirrhosis.
Colestyramine.
Cholestyramine (Questran) increases the excretion of bile acids, preventing their absorption. It is prescribed in a dose of 4 mg (mixed with water) during or after a meal; the daily dose is 12-16 mg. Colestyramine should be taken 1.5 hours before or after taking other drugs to avoid non-specific binding and reduce their absorption in the intestine. With a decrease in itching, the dose of colestyramine should be reduced to a minimum.