Primary biliary cholangitis

Primary biliary cholangitis (PBH) - chronic autoimmune liver disease, which is based on inflammation and destruction of the small bile ducts in the liver and is characterized by the presence in the blood of specific antibodies to the mitochondria of liver cells (AMA).

It mainly affects women – according to estimates, 1 in 1000 women in the world over 40 years of age is diagnosed with PBX, and the prevalence of this disease ranges from 1.9 to 40 per 100 thousand people.

Clinical manifestations

  • rapid fatigue, constant weakness
  • skin itching, especially at night (if skin diseases are excluded), leading to the appearance of scratching on the skin
  • deposits of cholesterol in the form of light plaques on the upper and lower eyelids (xanthelasma).


 Diagnostics

  • in the biochemical analysis of blood, an increase in a number of indicators is detected: alkaline phosphatase, GGTP, bilirubin, cholesterol, ALT, AST
  • autoantibodies are detected in the blood: antimitochondrial antibodies of the M2 type( AMA-M2), antinuclear antibodies (ANA)
  • the examination reveals an increase in the liver and spleen
  • a liver biopsy will help confirm the diagnosis


Treatment
There are currently no ways to completely cure this disease, however, it is possible to significantly ease the course of the disease and slow down the development of liver fibrosis, if you follow the following recommendations:

  • complete abstinence from alcohol
  • compliance with the daily routine-alternation of work and rest
  • diet: it is necessary to limit salt and foods rich in salt, foods high in calories and saturated fat (fatty meats, margarine, sweets)
  • drug therapy - constant intake of ursodeoxycholic acid preparations
  • regular monitoring of blood biochemical parameters (ALP, GGTP, bilirubin, ALT, AST)
  • observation of a hepatologist

Important:

  • The final diagnosis in some cases will help to establish only a puncture biopsy of the liver
  • Timely treatment will prevent the development of cirrhosis of the liver
  • It is necessary to take UDCA medications constantly
  • When treatment is discontinued, the symptoms of the disease resume
  • With late diagnosis and late start of treatment, there is a high risk of complications-cirrhosis of the liver, ascites, bleeding from the esophageal veins, encephalopathy
  • In this case, the only effective treatment is liver transplantation

Early access to a HEPATOLOGIST, strict compliance with the recommendations of the ATTENDING PHYSICIAN, the schedule of taking medications and monitoring laboratory parameters-the key to effective therapy!

GBUZ Moscow Clinical Scientific Center named after Loginov MHD