Clinical characteristics of primary sclerosing cholangitis. (A) The disease affects the biliary tree through inflammation and fibrosis, which results in multifocal biliary strictures and a high risk of cholangiocarcinoma. (B) When the disease progresses, high-grade strictures, often with prestenotic dilatation, can develop, leading to biliary obstruction and clinical symptoms. (C) Fatigue, itching, jaundice, bacterial cholangitis, and intermittent or persistent right upper-quadrant abdominal pain are common symptoms and signs in primary sclerosing cholangitis. Primary sclerosing cholangitis is associated with inflammatory bowel disease and 50–80% of affected individuals have concomitant inflammatory bowel disease. Primary sclerosing cholangitis inflammatory bowel disease shows distinct features from classic isolated inflammatory bowel disease, often with extensive colitis, a patchy inflammatory distribution that shows dominance of inflammation in the ascending colon, rectal sparing, and an increased risk of colorectal dysplasia compared with colitis without primary sclerosing cholangitis. (D) The inflammation almost always involves the colon and isolated ileal disease is rare. Reproduced with permission from Kari C Toverud.
Primary sclerosing cholangitis is a rare liver disease, characterised by biliary inflammation and fibrosis.
A new Seminar explores the clinical features, pathogenesis, and challenges and opportunities in managing the disease: spkl.io/63326A0F5O
