Primary sclerosing cholangitis
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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.[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.
DDx:
- Big.
Staging
Features:[7]
- 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
Notes:
- PSC often has minimal inflammation.[10]
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.
- ↑ 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.
- ↑ STC. 9 December 2010.