Abstract:
PURPOSE OF REVIEW:Primary sclerosing cholangitis (PSC) is a rare, idiopathic biliary disease often with an insidious onset, variable disease course, and premature death related to benign and malignant PSC-related sequelae. This review aims to discuss the epidemiology, clinical variants, and natural history of PSC, incorporating data from recent population-based studies. RECENT FINDINGS:PSC naturally leads to cirrhosis, cholangiocarcinoma, other hepatobiliary malignancies, dominant strictures, hepatic osteodystrophy, and bacterial cholangitis. The incidence of PSC appears to be increasing, the reasons for which are unclear. The time from diagnosis to liver transplant appears to be longer in more recent studies compared with earlier studies, suggesting a better overall prognosis than previously believed. In addition, with an increasing number of patients undergoing liver transplantation for PSC, the frequency of death because of liver failure has decreased, whereas cancer-related deaths have increased among patients with PSC. SUMMARY:PSC is a heterogeneous disease with a variety of clinical outcomes, both fatal and nonfatal. The progression of liver fibrosis in an individual patient is difficult to predict and may vary from a relatively benign, nonprogressive form to a rapidly progressive form with the need for liver transplantation.
journal_name
Curr Opin Gastroenteroljournal_title
Current opinion in gastroenterologyauthors
Takakura WR,Tabibian JH,Bowlus CLdoi
10.1097/MOG.0000000000000333subject
Has Abstractpub_date
2017-03-01 00:00:00pages
71-77issue
2eissn
0267-1379issn
1531-7056journal_volume
33pub_type
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journal_title:Current opinion in gastroenterology
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journal_title:Current opinion in gastroenterology
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journal_title:Current opinion in gastroenterology
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journal_title:Current opinion in gastroenterology
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journal_title:Current opinion in gastroenterology
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journal_title:Current opinion in gastroenterology
pub_type: 杂志文章,评审
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journal_title:Current opinion in gastroenterology
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