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.
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Clinical symptoms in the early stages of the disease
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The early signs of primary biliary cirrhosis (occur in more than 50% of cases) are:
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Itchy skin.
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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.
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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.
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Dry eyes.
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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).
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Clinical symptoms in the late stage of the disease
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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:
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Ascites.
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Spider veins.
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Hepatic failure and encephalopathy .
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In 10% of patients around the eyes and on extensor surfaces of the joints, xanthelasma and xanthomas are formed.
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Xanthelasma (yellow, slightly rising plaques around the eyelids).
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In 25% of cases, hyperpigmentation of the skin is detected.
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In 10% of cases, jaundice occurs .
ellowness sclera.
Jaundice - the skin becomes yellow.
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In 50-75% of patients, Sjogren's syndrome develops, the main manifestations of which are: xerophthalmia (dry eyes), xerostomia (dry mouth).
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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).
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Medical history
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
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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.