Difference between revisions of "Primary sclerosing cholangitis"
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*IgG4-associated cholangitis - see [[IgG4-related | *IgG4-associated cholangitis - see [[IgG4-related disease]].<ref name=pmid19633647>{{Cite journal | last1 = Deshpande | first1 = V. | last2 = Sainani | first2 = NI. | last3 = Chung | first3 = RT. | last4 = Pratt | first4 = DS. | last5 = Mentha | first5 = G. | last6 = Rubbia-Brandt | first6 = L. | last7 = Lauwers | first7 = GY. | title = IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. | journal = Mod Pathol | volume = 22 | issue = 10 | pages = 1287-95 | month = Oct | year = 2009 | doi = 10.1038/modpathol.2009.94 | PMID = 19633647 }}</ref> | ||
*Others. | *Others. | ||
Revision as of 01:06, 24 March 2015
Primary sclerosing cholangitis, abbreviated PSC, is an uncommon medical liver disease that can afflicts the young and old, and is often associated with ulcerative colitis.
Pericholangitis is considered a synonym for small duct PSC.[1]
General
- Strongly associated with ulcerative colitis; 75-90% of PSC patients have inflammatory bowel disease (IBD).[2]
- Risk for cholangiocarcinoma.[3]
Serology:
- p-ANCA (MPO-ANCA) +ve in ~ 90% of cases.[4]
Diagnosis
- Diagnosed radiologically.
- Classically described as a chain of lakes.
- Liver biopsy is rarely useful diagnostically[5] - 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.[6]
- Not pathognomonic for PSC[6] - but not too much else looks like this on microscopy (ergo good fellowship exam question).
- +/-Ductopenia.
- +/-Ductal proliferation.
Notes:
- PSC often has minimal inflammation.[7]
DDx:
- IgG4-associated cholangitis - see IgG4-related disease.[8]
- Others.
Staging
Features:[9]
- 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
See also
References
- ↑ URL: http://emedicine.medscape.com/article/181889-overview. Accessed on: 25 January 2012.
- ↑ Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: http://emedicine.medscape.com/article/187724-overview. Accessed on: 29 November 2009.
- ↑ Jesudian, AB.; Jacobson, IM. (2009). "Screening and diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis.". Rev Gastroenterol Disord 9 (2): E41-7. PMID 19668124.
- ↑ Terjung, B.; Worman, HJ. (Aug 2001). "Anti-neutrophil antibodies in primary sclerosing cholangitis.". Best Pract Res Clin Gastroenterol 15 (4): 629-42. doi:10.1053/bega.2001.0209. PMID 11492972.
- ↑ Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: http://emedicine.medscape.com/article/187724-diagnosis. Accessed on: 29 November 2009.
- ↑ 6.0 6.1 Steele et al. URL: http://www.medscape.com/viewarticle/552500_6. Accessed on: 29 November 2009.
- ↑ STC. 9 December 2010.
- ↑ Deshpande, V.; Sainani, NI.; Chung, RT.; Pratt, DS.; Mentha, G.; Rubbia-Brandt, L.; Lauwers, GY. (Oct 2009). "IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material.". Mod Pathol 22 (10): 1287-95. doi:10.1038/modpathol.2009.94. PMID 19633647.
- ↑ Steele et al. URL: http://www.medscape.com/viewarticle/552500_6. Accessed on: 29 November 2009.
- ↑ URL: http://trialx.com/curebyte/2011/07/08/clinical-trials-and-images-of-primary-sclerosing-cholangitis/. Accessed on: 1 January 2012.
- ↑ URL: http://www.onmedica.com/NewsArticle.aspx?id=d7f992b5-6dee-46c6-8383-bbdfb4528ccc. Accessed on: 1 January 2011.