Primary sclerosing cholangitis

Description:

The Primary Sclerosing Cholangitis (PSC) – the state which is relating to group of autoimmune diseases of a liver, characterized by development of antibodies to bilious channels with bile outflow disturbance

Symptoms of Primary sclerosing cholangitis:

As a rule, the debut of a disease happens without bright symptoms and the first manifestation of PSH are biochemical changes (increase in activity of an alkaline phosphatase, serumal transaminases). Even at an asymptomatic current the disease can progress with development of cirrhosis and portal hypertensia.

Usually at the beginning of a disease weight loss, increased fatigue, pain in right hypochondrium, a skin itch, passing jaundice is noted. Existence of symptoms demonstrates far come process. Fever is, as a rule, not characteristic though cases when the fever and fever act on the first place and in maintenance with jaundice meet, an itch and pains in hypochondrium imitate an acute bacterial cholangitis.

Diagnosis of a disease.  At a research of blood serum increase in an alkaline phosphatase, bilirubin, level of γ-globulins is noted, IgM, appear high antiserum capacities to smooth muscles and antinuclear antibodies.

At ultrasonography a research reveal a thickening of walls of bilious channels.

Choice method for diagnosis of PSH is the holangiografiya at which sites of promiseds and expanded hepatic channels are visible.

Reasons of Primary sclerosing cholangitis:

The origin of primary sclerosing cholangitis remains to the unknown. The most probable is the assumption about presence of the infectious agent, being a provocative factor at genetically and immunno predisposed people.

Men are ill twice more often than women. The disease usually develops at the age of 25-45 years though it is possible also at children of early age.

In 70% of cases patients have an accompanying nonspecific ulcer colitis (a chronic autoimmune disease with involvement of a mucous membrane of a large intestine), there can also be a combination and to other autoimmune states (a thyroiditis, a diabetes mellitus of the I type).

Treatment of Primary sclerosing cholangitis:

Effective pathogenetic treatment of primary sclerosing cholangitis does not exist. Reception of ursodezoksikholiyevy acid (UDKH) improves biochemical indicators and reduces activity of a disease according to a liver biopsy. In certain cases it is necessary to appoint the drugs suppressing activity of immune system – corticosteroids and cytostatics. Completion of deficit of calcium, vitamin D and other fat-soluble vitamins is necessary.