Difference between revisions of "Primary sclerosing cholangitis"

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#redirect [[Medical_liver_disease#Primary_sclerosing_cholangitis]]
'''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]]

Revision as of 05:01, 18 September 2014

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

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

  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. ↑ Steele et al. URL: http://www.medscape.com/viewarticle/552500_6. Accessed on: 29 November 2009.
  8. ↑ URL: http://trialx.com/curebyte/2011/07/08/clinical-trials-and-images-of-primary-sclerosing-cholangitis/. Accessed on: 1 January 2012.
  9. ↑ URL: http://www.onmedica.com/NewsArticle.aspx?id=d7f992b5-6dee-46c6-8383-bbdfb4528ccc. Accessed on: 1 January 2011.
  10. ↑ STC. 9 December 2010.
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