Cholangitis, primary sclerosing

Cholangitis, primary sclerosing

Primary sclerosing cholangitis – progressive sclerosis of the bile ducts, leading to their stenosis or obstruction. Diagnosed, as a rule, by the age of 30-40 (with recessive forms much earlier). 90% of cases are women. Etiology and pathogenesis. Immune shifts are characteristic, glycoproteins HLA-B8, HLA-DR3, HLA-DRw52a are found in patients. Several inherited forms are known

    • Primary biliary cirrhosis (109720, dihydrolipoamide acetyltransferase deficiency [EC, ED])
    • Multifocal fibrosclerosis (228800, p). Mediastinal and retroperitoneal fibrosis, Rydel’s thyroiditis were also found.
    • Immunodeficiency form (242850, p), predominantly with IgM deficiency
    • Pancreatitis, sclerosing cholangitis and dry syndrome (260480, p). Hepatomegaly, dry keratoconjunctivitis, dry mucous membranes are also characteristic.
    • Primary biliary cirrhosis is often combined with Sjögren’s syndrome (75% of patients), the presence of antithyroid antibodies (25% of patients), rheumatoid arthritis (5% of patients), and CREST syndrome.

Clinical picture

    • Symptoms develop gradually, usually over several years
    • Pain in the right upper quadrant of the abdomen
    • Jaundice (possible without pain attack, chills and fever)
    • Skin itching
    • In the later stages of the disease, liver failure, portal hypertension, ascites develop.
    • In 30% of patients, concomitant inflammatory diseases of the gastrointestinal tract (especially Crohn’s disease, ulcerative colitis) are detected.


    • Significant increase in serum ALP activity
    • Ultrasound – wall thickening and stenosis over a large extent of the bile ducts
    • Endoscopic retrograde cholangiography or percutaneous transhepatic cholangiography visualizes multiple, brief areas of stenosis or saccular dilatations along the intra- and extrahepatic ducts—beaded appearance
    • Liver biopsy reveals obliteration of the bile ducts, periductal fibrosis, inflammation, and disappearance of the bile ducts.


    • Vitamin D and calcium in the diet. As food additives, the use of triglycerides with an average chain length is likely (found in small amounts in butter, coconut oils, etc.)
    • To relieve itching – cholestyramine 4-6 g / day in 2 doses (binds bile salts in the intestine)
    • With hypoprothrombinemia – vitamin K ((phytomenadione) 5-10 mg / essence / day 2-3 days
    • Glucocorticoids, azathioprine, chlorambucil (chlorbutine), colchicine and methotrexate (effectiveness varies, many side effects)
    • Ursodeoxycholic acid (ursofalk) in unopened cases.

Surgery. The goal is to restore a constant adequate passage of bile into the intestine. The type of intervention depends on the level of damage to the biliary tract and the degree of sclerosis

    • Internal drainage of the bile ducts by anastomosis between the hepatic (common bile) duct and the small intestine is the operation of choice. However, it gives good results only with a predominant lesion of the extrahepatic bile ducts.
    • External drainage of the bile ducts with the help of a T-shaped drainage provides an adequate outflow of bile in the early postoperative period. Later, infection of the drainage with the development of bacterial cholangitis is likely
    • Cholecystectomy is performed only in the presence of pathological changes in the gallbladder
    • Liver transplantation (5-year survival rate – 70%).

The prognosis is uncertain. Cholangiocarcinoma develops in 10–15% of patients. ICD. K83.0 Cholangitis

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