Difference between revisions of "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]].
'''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''.<ref>URL: [http://emedicine.medscape.com/article/181889-overview http://emedicine.medscape.com/article/181889-overview]. Accessed on: 25 January 2012.</ref>
'''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>

Revision as of 05:03, 18 September 2014

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

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:

  • Big.

Staging

Features:[8]

  • 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

  1. ↑ URL: http://emedicine.medscape.com/article/181889-overview. Accessed on: 25 January 2012.
  2. ↑ Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: http://emedicine.medscape.com/article/187724-overview. Accessed on: 29 November 2009.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ Khurana V, Singh T. Primary sclerosing cholangitis. eMedicine.com. URL: http://emedicine.medscape.com/article/187724-diagnosis. Accessed on: 29 November 2009.
  6. ↑ 6.0 6.1 Steele et al. URL: http://www.medscape.com/viewarticle/552500_6. Accessed on: 29 November 2009.
  7. ↑ STC. 9 December 2010.
  8. ↑ Steele et al. URL: http://www.medscape.com/viewarticle/552500_6. Accessed on: 29 November 2009.
  9. ↑ URL: http://trialx.com/curebyte/2011/07/08/clinical-trials-and-images-of-primary-sclerosing-cholangitis/. Accessed on: 1 January 2012.
  10. ↑ URL: http://www.onmedica.com/NewsArticle.aspx?id=d7f992b5-6dee-46c6-8383-bbdfb4528ccc. Accessed on: 1 January 2011.
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