Primary sclerosing cholangitis - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Primary sclerosing cholangitis is a cholestatic liver disease causing continued destruction of the bile ducts, cirrhosis, and end-stage liver disease.
Predominantly affects young and middle-aged men, often with underlying inflammatory bowel disease.
Patients may be asymptomatic at diagnosis but develop symptoms of pruritus and jaundice.
Diagnosis involves laboratory tests and cholangiography.
No effective medical therapy is available.

Typical endoscopic retrograde cholangiopancreatography findings in a patient with PSC: multifocal strictures of the intra- and extrahepatic bile ducts

Dr Kris Kowdley's collection

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Definition

Epidemiology

Etiology

Pathophysiology

Images

Typical endoscopic retrograde cholangiopancreatography findings in a patient with PSC: multifocal strictures of the intra- and extrahepatic bile ducts
Normal endoscopic retrograde cholangiopancreatography
Normal cholangiogram-pancreatogram

Citations

Key Articles

European Association for the Study of the Liver. EASL clinical practice guidelines on sclerosing cholangitis. J Hepatol. 2022 Sep;77(3):761-806.[Abstract][Full Text]
Bowlus CL, Arrivé L, Bergquist A, et al. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology. 2023 Feb 1;77(2):659-702.[Abstract][Full Text]
Chapman R, Fevery J, Kalloo A, et al. Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010 Feb;51(2):660-78.[Abstract][Full Text]
Dave M, Elmunzer BJ, Dwamena BA, et al. Primary sclerosing cholangitis: meta-analysis of diagnostic performance of MR cholangiopancreatography. Radiology. 2010 Aug;256(2):387-96.[Abstract]
LaRusso NF, Shneider BL, Black D, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology. 2006 Sep;44(3):746-64.[Abstract]
Poropat G, Giljaca V, Stimac D, et al. Bile acids for primary sclerosing cholangitis. Cochrane Database Syst Rev. 2011 Jan 19;(1):CD003626.[Abstract][Full Text]

Other Online Resources

Mayo Clinic: revised natural history model for primary sclerosing cholangitis

Referenced Articles

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