Small Duct Primary Sclerosing Cholangitis: An Underdiagnosed Cause of Chronic Liver Disease and Cirrhosis
Open Access
- 17 March 2020
- journal article
- research article
- Published by Springer Science and Business Media LLC in Cureus
- Vol. 12 (3), e7298
- https://doi.org/10.7759/cureus.7298
Abstract
Classic or large duct primary sclerosing cholangitis (PSC) is part of the PSC spectrum. It is diagnosed on clinical and biochemical findings of cholestasis supported by biliary tree changes on cholangiography, forgoing the need for an invasive liver biopsy. The spectrum contains various PSC variants with distinct clinical courses and outcomes. We present a case of small duct PSC, a rare variant that manifested insidiously with clinical and objective cholestasis but appeared negative on diagnostic cholangiography. Eventually, a liver biopsy was obtained that revealed chronic bilious disease of the small and microscopic ducts with simultaneous changes consistent with liver cirrhosis. Despite presenting like its classical counterpart, small duct PSC can remain undetectable on cholangiography due to the diminutive size of the bile ducts requiring histological confirmation. In contrast to classic PSC, small duct PSC portends a much better prognosis. However, it eventually progresses to the classic form or end-stage liver disease, requiring patients to receive timely surveillance and transplantation referrals. Due to the limited understanding of this disease process, patients with similar presentations often pose a diagnostic dilemma due to the clinical and cholangiographic mismatch. This case aims to reaffirm that a negative cholangiography does not rule out the PSC spectrum and that small duct disease is a rare but growing entity. The paucity in cases emphasizes the importance of isolated reports in guiding workup and management, especially since surveillance schedules and transplantation guidelines have not been formally established.This publication has 20 references indexed in Scilit:
- Incidence, prevalence, and natural history of primary sclerosing cholangitis in the United KingdomMedicine, 2017
- The evolution of natural history of primary sclerosing cholangitisCurrent Opinion in Gastroenterology, 2017
- The Natural History of Primary Sclerosing Cholangitis in ChildrenJournal of Pediatric Gastroenterology and Nutrition, 2016
- Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in swedenHepatology, 2010
- Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel diseaseWorld Journal of Gastroenterology, 2008
- Impact of inflammatory bowel disease and ursodeoxycholic acid therapy on small-duct primary sclerosing cholangitisHepatology, 2007
- The Burden of Large and Small Duct Primary Sclerosing Cholangitis in Adults and Children: A Population-Based AnalysisThe American Journal of Gastroenterology, 2007
- PSC-IBD: a unique form of inflammatory bowel disease associated with primary sclerosing cholangitisGut, 2005
- Incidence, clinical spectrum, and outcomes of primary sclerosing cholangitis in a united states communityGastroenterology, 2003
- Small-duct primary sclerosing cholangitis: A long-term follow-up studyHepatology, 2002