Review article

Diagnosis and treatment of primary sclerosing cholangitis

Armin Mehmedović ; Klinički bolnički centar "Sestre milosrdnice", Klinika za unutarnje bolesti, Zavod za gastroenterologiju i hepatologiju
Marko Nikolić ; Klinički bolnički centar "Sestre milosrdnice", Klinika za unutarnje bolesti, Zavod za gastroenterologiju i hepatologiju
Dražen Zekanović ; Opća bolnica Zadar, Služba za internu medicinu, Odjel za kardiologiju; Sveučilište u Zadru, Odjel za zdravstvene studije
Jadranko Turčinov ; Opća bolnica Zadar, Služba za internu medicinu, Odjel za gastroenterologiju
Neven Ljubičić ; Klinički bolnički centar "Sestre milosrdnice", Klinika za unutarnje bolesti, Zavod za gastroenterologiju i hepatologiju

Abstract

Primary sclerosing cholangitis (PSC) is a chronic, immune-mediated liver disease. It is characterized by chronic inflammation and fibrosis of intra- and extrahepatic bile ducts. In the course of 10 to 15 years, in most patients the disease leads to decompensated liver cirrhosis due to cholestasis and progressive liver fibrosis. The incidence of primary sclerosing cholangitis is 1 per 100, 000 population. It is estimated that 60% to 80% patients with PSC have inflammatory bowel disease. The etiology of the disease is unclear. At the time of diagnosis, most patients are asymptomatic and only abnormal liver tests and/or the discovery of dilated bile ducts on imaging findings indicate that the disease is present. In symptomatic patients, fatigue and pruritus are the most common symptoms at disease onset. As the disease advances, jaundice, weight loss and abdominal pain occur. The gold standard in diagnosing PSC is magnetic resonance cholangiopancreatography (MRCP), which replaced endoscopic retrograde cholangiopancreatography (ERCP).
PSC is treated with ursodeoxycholic acid, ERCP interventions and liver transplant. PSC is a slowly progressive disease and most patients die within 10 to 12 years from the time when the disease was diagnosed, if they do not receive a liver transplant. A significant number of patients develop hepatobiliary or colorectal cancer.

Keywords

primary sclerosing cholangitis; magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography; liver cirrhosis; liver transplant

Hrčak ID:

197943

URI

https://hrcak.srce.hr/197943

Publication date:

27.3.2018.

Article data in other languages: croatian

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