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Primary sclerosing cholangitis (view source)
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'''Primary sclerosing cholangitis''', abbreviated '''PSC''', is an uncommon [[medical liver disease]] that can afflicts the young or old, and is often associated with [[ulcerative colitis]]. | |||
'''Pericholangitis''' is considered a synonym for ''small duct PSC''.<ref>URL: [http://emedicine.medscape.com/article/181889-overview http://emedicine.medscape.com/article/181889-overview]. Accessed on: 25 January 2012.</ref> | |||
==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> | |||
*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> | |||
Serology: | |||
*p-ANCA (MPO-ANCA) +ve in ~ 90% of cases.<ref name=pmid11492972>{{Cite journal | last1 = Terjung | first1 = B. | last2 = Worman | first2 = HJ. | title = Anti-neutrophil antibodies in primary sclerosing cholangitis. | journal = Best Pract Res Clin Gastroenterol | volume = 15 | issue = 4 | pages = 629-42 | month = Aug | year = 2001 | doi = 10.1053/bega.2001.0209 | PMID = 11492972 }}</ref> | |||
===Diagnosis=== | |||
*'''Diagnosed radiologically'''. | |||
**Classically described as a ''chain of lakes''. | |||
*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. | |||
===Treatment=== | |||
*None very good. | |||
*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> | |||
**Not pathognomonic for PSC<ref name=medscape_psc/> - but not too much else looks like this on microscopy (ergo good fellowship exam question). | |||
*+/-Ductopenia. | |||
*+/-Ductal proliferation. | |||
DDx: | |||
*Big. | |||
===Staging=== | |||
Features:<ref>Steele et al. URL: [http://www.medscape.com/viewarticle/552500_6 http://www.medscape.com/viewarticle/552500_6]. Accessed on: 29 November 2009.</ref> | |||
*Stage I - focal portal inflammation, +/- duct abnormalities, no fibrosis. | |||
*Stage II - portal enlargement (fibrosis), +/- inflammation. | |||
*Stage III - bridging fibrosis + [[necrosis]]. | |||
*Stage IV - cirrhosis. | |||
Notes: | |||
*Similar to PBC staging. | |||
===Images=== | |||
*[http://trialx.com/curetalk/wp-content/blogs.dir/7/files/2011/05/diseases/Primary_Sclerosing_Cholangitis-2.jpg PSC (trialx.com)].<ref>URL: [http://trialx.com/curebyte/2011/07/08/clinical-trials-and-images-of-primary-sclerosing-cholangitis/ http://trialx.com/curebyte/2011/07/08/clinical-trials-and-images-of-primary-sclerosing-cholangitis/]. Accessed on: 1 January 2012.</ref> | |||
*[http://www.onmedica.com/getresource.aspx?resourceid=e398a3f2-e030-40e6-823b-2e8e2cbbe4bb PSC (onmedica.com)].<ref>URL: [http://www.onmedica.com/NewsArticle.aspx?id=d7f992b5-6dee-46c6-8383-bbdfb4528ccc http://www.onmedica.com/NewsArticle.aspx?id=d7f992b5-6dee-46c6-8383-bbdfb4528ccc]. Accessed on: 1 January 2011.</ref> | |||
Notes: | |||
*PSC often has minimal inflammation.<ref>STC. 9 December 2010.</ref> | |||
==See also== | |||
*[[Medical liver disease]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Medical liver disease]] | |||