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==Primary sclerosing cholangitis== | ==Primary sclerosing cholangitis== | ||
*Abbreviated ''PSC''. | *Abbreviated ''PSC''. | ||
===General=== | |||
*Strongly associated with [[ulcerative colitis]]; 75-90% of PSC patients have [[inflammatory bowel disease]] (IBD).<ref name=emed_psc_over>Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: [http://emedicine.medscape.com/article/187724-overview http://emedicine.medscape.com/article/187724-overview]. Accessed on: 29 November 2009.</ref> | *Strongly associated with [[ulcerative colitis]]; 75-90% of PSC patients have [[inflammatory bowel disease]] (IBD).<ref name=emed_psc_over>Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: [http://emedicine.medscape.com/article/187724-overview http://emedicine.medscape.com/article/187724-overview]. Accessed on: 29 November 2009.</ref> | ||
*Risk for [[cholangiocarcinoma]].<ref name=pmid19668124>{{Cite journal | last1 = Jesudian | first1 = AB. | last2 = Jacobson | first2 = IM. | title = Screening and diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis. | journal = Rev Gastroenterol Disord | volume = 9 | issue = 2 | pages = E41-7 | month = | year = 2009 | doi = | PMID = 19668124 }}</ref> | *Risk for [[cholangiocarcinoma]].<ref name=pmid19668124>{{Cite journal | last1 = Jesudian | first1 = AB. | last2 = Jacobson | first2 = IM. | title = Screening and diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis. | journal = Rev Gastroenterol Disord | volume = 9 | issue = 2 | pages = E41-7 | month = | year = 2009 | doi = | PMID = 19668124 }}</ref> | ||
===Diagnosis=== | ====Diagnosis==== | ||
*Diagnosed radiologically. | *'''Diagnosed radiologically'''. | ||
*Liver biopsy is rarely useful diagnostically<ref name=emed_psc>Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: [http://emedicine.medscape.com/article/187724-diagnosis http://emedicine.medscape.com/article/187724-diagnosis]. Accessed on: 29 November 2009.</ref> - as the disease may be patchy. | *Liver biopsy is rarely useful diagnostically<ref name=emed_psc>Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: [http://emedicine.medscape.com/article/187724-diagnosis http://emedicine.medscape.com/article/187724-diagnosis]. Accessed on: 29 November 2009.</ref> - as the disease may be patchy. | ||
**The utility of the biopsy is staging. | **The utility of the biopsy is staging. | ||
===Treatment=== | ====Treatment==== | ||
*None very good. | *None very good. | ||
*May be indication for transplant. | *May be indication for transplant. | ||
=== | ===Microscopic=== | ||
Features: | |||
*Classic: "onion-skinning" - cells layer around the bile ducts; "onion skin" present in approx. 40% of cases.<ref name=medscape_psc>Steele et al. URL: [http://www.medscape.com/viewarticle/552500_6 http://www.medscape.com/viewarticle/552500_6]. Accessed on: 29 November 2009.</ref> | *Classic: "onion-skinning" - cells layer around the bile ducts; "onion skin" present in approx. 40% of cases.<ref name=medscape_psc>Steele et al. URL: [http://www.medscape.com/viewarticle/552500_6 http://www.medscape.com/viewarticle/552500_6]. Accessed on: 29 November 2009.</ref> | ||
**Not pathognomonic for PSC<ref name=medscape_psc/> - but not too much else looks like this on microscopy (ergo good fellowship exam question). | **Not pathognomonic for PSC<ref name=medscape_psc/> - but not too much else looks like this on microscopy (ergo good fellowship exam question). | ||
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Micrographs: | Micrographs: | ||
*[http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675%2806%2970838-5&figureId=fig1 Onion-skinning (pathconsultddx.com)] | *[http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675%2806%2970838-5&figureId=fig1 Onion-skinning (pathconsultddx.com)] | ||
Notes: | |||
*PSC often has minimal inflammation.<ref>STC. 9 December 2010.</ref> | |||
====Staging==== | ====Staging==== |
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