Primary sclerosing cholangitis (PSC) is a male-predominant autoimmune-mediated inflammatory disease of the liver that affects both the large and small bile ducts that eventually progresses to cirrhosis, portal hypertension, and liver failure. PSC patients are usually asymptomatic at an early stage. About 80% of PSC patients also developed inflammatory bowel disease (IBD)—a majority of them have ulcerative colitis. Other PSC-IBD patients may have Crohn’s disease. The accurate diagnosis of PSC is based on clinical history, liver biochemistry, and imaging with magnetic resonance imaging with cholangiopathy (MRI/MRCP) and endoscopic retrograde cholangiopancreatography (ERCP). A liver biopsy is performed when imaging results are indeterminate. A liver biopsy helps to differentiate small duct PSC from overlapping autoimmune hepatitis or IgG4 cholangiopathy. Although perinuclear antineutrophilic cytoplasmic antibodies, anti-nuclear antibodies, anti-muscle antibodies, and IgG4 may be detected, there are no disease-specific autoantibodies in PSC. PSC patients have a higher risk of developing cholangiocarcinoma, and cholangiocarcinoma surveillance is recommended. Since there is no effective cure for PSC, current treatments are directed at alleviating symptoms and improving quality of life. A liver transplant is necessary for advanced-stage PSC patients. Colon cancer prevention with an annual colonoscopy is recommended for PSC-IBD patients.
Hsu, R., Leung, P., Invernizzi, P., Gershwin, M. (2025). Primary Sclerosing Cholangitis. In Y. Shoenfeld, R. Cervera, G. Espinosa, M.E. Gershwin (a cura di), Autoimmune Disease Diagnosis: Systemic and Organ-specific Diseases, Second Edition (pp. 433-439). Springer Nature [10.1007/978-3-031-69895-8_61].
Primary Sclerosing Cholangitis
Invernizzi P.;
2025
Abstract
Primary sclerosing cholangitis (PSC) is a male-predominant autoimmune-mediated inflammatory disease of the liver that affects both the large and small bile ducts that eventually progresses to cirrhosis, portal hypertension, and liver failure. PSC patients are usually asymptomatic at an early stage. About 80% of PSC patients also developed inflammatory bowel disease (IBD)—a majority of them have ulcerative colitis. Other PSC-IBD patients may have Crohn’s disease. The accurate diagnosis of PSC is based on clinical history, liver biochemistry, and imaging with magnetic resonance imaging with cholangiopathy (MRI/MRCP) and endoscopic retrograde cholangiopancreatography (ERCP). A liver biopsy is performed when imaging results are indeterminate. A liver biopsy helps to differentiate small duct PSC from overlapping autoimmune hepatitis or IgG4 cholangiopathy. Although perinuclear antineutrophilic cytoplasmic antibodies, anti-nuclear antibodies, anti-muscle antibodies, and IgG4 may be detected, there are no disease-specific autoantibodies in PSC. PSC patients have a higher risk of developing cholangiocarcinoma, and cholangiocarcinoma surveillance is recommended. Since there is no effective cure for PSC, current treatments are directed at alleviating symptoms and improving quality of life. A liver transplant is necessary for advanced-stage PSC patients. Colon cancer prevention with an annual colonoscopy is recommended for PSC-IBD patients.
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