Primary sclerosing cholangitis
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 systemic 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.