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Primary sclerosing cholangitis
Last reviewed: 23.04.2024
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Primary sclerosing cholangitis is a chronic cholestatic syndrome characterized by inhomogeneous inflammation, fibrosis and intra- and extrahepatic strictures of the biliary tract. Eighty percent of patients have inflammatory bowel disease, most often ulcerative colitis. Symptoms of fatigue and skin itching develop late. Diagnosis is based on contrasting cholangiography (ERCP) or magnetic resonance cholangiopancreatography. The disease ultimately leads to obliteration of the bile ducts with the development of liver cirrhosis, hepatic insufficiency and sometimes cholangiocarcinoma. When the disease progresses, liver transplantation is indicated.
[What causes primary sclerosing cholangitis?
The reason is unknown. However, the primary sclerosing cholangitis (PSC) is closely related to inflammatory bowel disease; approximately 5% of patients have ulcerative colitis and approximately 1% have Crohn's disease. This association and the presence of certain autoantibodies [eg, anti-smooth muscle and perinuclear antineutrophil antibodies (pANCA)] suggest immunomodulatory mechanisms. Apparently, T-lymphocytes are involved in the damage of the bile ducts, which indicates violations in the cell link of immunity. Genetic predisposition is indicated by family history and a higher incidence among people with HLA B8 and HLA DR3, which often correlate with autoimmune diseases. Unproved trigger factors (eg, bacterial infection or ischemic duct damage) are likely to cause the development of primary sclerosing cholangitis in genetically predisposed patients. Sclerosing cholangitis in HIV-infected patients may be cryptogenic or caused by cytomegalovirus.
Symptoms of primary sclerosing cholangitis
The average age at diagnosis is 40 years; 70% of patients are men. The onset of the disease is usually gradual, with no apparent clinical symptoms, with progressive malaise and skin pruritus. Jaundice, as a rule, develops later. Repeated episodes of pain in the right side of the abdomen and fever, possibly due to an upward bacterial infection of the biliary tract, are observed in 10-15% of patients with a clinical manifestation of the disease, typical pain in the right side. There may be steatorrhoea and signs of deficiency of fat-soluble vitamins. Persistent harbingers of jaundice characterize the progression of the disease. Clinically manifested gallstones and choledocholithiasis are usually formed in about one-third of patients. In some patients, the disease is asymptomatic for a long time, manifested first by hepatomegaly or cirrhosis of the liver. The terminal phase of the disease includes decompensated liver cirrhosis, portal hypertension, ascites and liver failure.
Despite the association of primary sclerosing cholangitis with inflammatory bowel diseases, both these diseases usually occur separately. Ulcerative colitis may appear several years before the primary sclerosing cholangitis, but usually its more relaxed course is associated with primary sclerosing cholangitis. The presence of both diseases increases the risk of developing colorectal cancer, regardless of whether liver transplantation was performed in the primary sclerosing cholangitis. Similarly, total colectomy does not alter the course of primary sclerosing cholangitis. Cholangiocarcinoma develops in 10-15% of patients with primary sclerosing cholangitis.
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Diagnosis of primary sclerosing cholangitis
Primary sclerosing cholangitis is suspected in patients with unexplained changes in functional hepatic tests; if the patient suffers an inflammatory bowel disease, the suspicion of primary sclerosing cholangitis increases. Typical biochemical disorders characteristic of hepatic cholestasis, the level of alkaline phosphatase and gamma-glutamyltransferase usually increases more significantly than the level of aminotransferase. IgG and IgM levels are generally elevated, and the tests for anti-smooth muscle antibodies and pANCA are usually positive. The test for antimitochondrial antibodies, positive for primary biliary cirrhosis, is negative.
The examination of the hepatobiliary system usually begins with ultrasound to exclude extrahepatic biliary obstruction. Diagnosis of primary sclerosing cholangitis requires the detection of multiple strictures and extensions involving intra- and extrahepatic bile ducts-cholangiography is performed for this purpose (ultrasound can only assume their presence). Direct cholangiography (for example, ERCP) is the "gold standard"; However, magnetic resonance cholangiopancreatography (MZHPG) provides a better image and becomes the main alternative non-invasive diagnostic method. To verify the diagnosis of liver biopsy, as a rule, is not required. If the biopsy is performed according to other indications, then it shows proliferation of bile ducts, periductal fibrosis, inflammation and obliteration of the bile ducts. In the case of progression of the disease, fibrosis spreads from the portal zones and, ultimately, leads to biliary cirrhosis of the liver.
A dynamic examination using ERCP and a biopsy brush for cytology will help in predicting the development of cholangiocarcinoma.
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Treatment of primary sclerosing cholangitis
In some patients, the disease can be asymptomatic for several years, despite the progressive changes. The time interval from the verification of the diagnosis to the development of liver failure may be about 12 years.
Asymptomatic flow generally requires only observation and monitoring (for example, physical examination and functional liver tests 2 times a year). The use of ursodeoxycholic acid can reduce itching and improve biochemical markers. Chronic cholestasis and cirrhosis of the liver require treatment. With recurrent bacterial cholangitis, antibacterial therapy and ERCP are performed according to indications.
Prognosis of primary sclerosing cholangitis
If the dominant stricture is detected (approximately 20%), endoscopic dilatation is required to reduce symptoms and a cytological study to exclude the tumor. Any infection (eg, cryptosporidiosis, cytomegalovirus) is treatable.
Liver transplantation is the only method of treatment that increases the duration of life in idiopathic primary sclerosing cholangitis - can lead to a cure. Recurrent bacterial cholangitis or complications at the terminal stage of liver disease, such as severe ascites, portosystemic encephalopathy, bleeding from varicose veins of the esophagus, are indications for liver transplantation.