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Primary biliary cholangitis (view source)
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'''Primary biliary cirrhosis''', abbreviated '''PBC''', is a rare [[medical liver disease]]. | |||
==General== | |||
Epidemiology: | |||
*Female>male (~9:1).<ref>{{Ref DCHH|162}}</ref> | |||
*Usually middle age. | |||
*Associated with other autoimmune conditions ([[Sjögren syndrome]], progressive systemic sclerosis, [[celiac disease]]). | |||
Etiology: | |||
*Autoimmune. | |||
Serology: | |||
*AMA +ve.<ref name=pmid20955967>{{Cite journal | last1 = Nguyen | first1 = DL. | last2 = Juran | first2 = BD. | last3 = Lazaridis | first3 = KN. | title = Primary biliary cirrhosis. | journal = Best Pract Res Clin Gastroenterol | volume = 24 | issue = 5 | pages = 647-54 | month = Oct | year = 2010 | doi = 10.1016/j.bpg.2010.07.006 | PMID = 20955967 }}</ref> | |||
Classic presentation: | |||
*Pruritis. | |||
Pathophysiology: | |||
*Septal bile duct attacked. | |||
Treatment: | |||
*Ursodeoxycholic acid. | |||
*May be indication for transplant. | |||
==Microscopic== | |||
Features: | |||
*"Florid duct lesion":<ref name=pmid7905494>{{Cite journal | last1 = Nakanuma | first1 = Y. | last2 = Harada | first2 = K. | title = Florid duct lesion in primary biliary cirrhosis shows highly proliferative activities. | journal = J Hepatol | volume = 19 | issue = 2 | pages = 216-21 | month = Sep | year = 1993 | doi = | PMID = 7905494 }}</ref> | |||
**Intraepithelial lymphocytes - in bile duct - '''key feature'''. | |||
**Bile duct epithelial cells with eosinophilic cytoplasm.<ref>OA. 11 September 2009.</ref> | |||
*Plasma cells. | |||
*[[Granulomas]] - close to bile duct. | |||
**Seen in classic presentation -- often not present or poorly formed. | |||
*Focal damage (may be missed on biopsy -- due to sampling). | |||
*"Garland" cirrhosis -- has irregular border (unlike in EtOH). | |||
**''Garland'' originally "wreath of flowers" (in French).<ref>[http://dictionary.reference.com/browse/garland http://dictionary.reference.com/browse/garland]</ref> | |||
Notes: | |||
*[[PAS stain]] useful for examining basement membrane... which is lost in PBC. | |||
*Lobular inflammation should be minimal. | |||
*May cause [[cholestasis|cholestatic picture]].<ref name=pmid21452140>{{Cite journal | last1 = Grimm | first1 = D. | last2 = Thimme | first2 = R. | title = [Cholestatic liver diseases]. | journal = Ther Umsch | volume = 68 | issue = 4 | pages = 195-9 | month = Apr | year = 2011 | doi = 10.1024/0040-5930/a000150 | PMID = 21452140 }}</ref> | |||
DDx:<ref name=Ref_DCHH163>{{Ref DCHH|163}}</ref> | |||
*[[Sarcoidosis]] (if granulomas present). | |||
*[[Primary sclerosing cholangitis]]. | |||
*Viral hepatitis. | |||
*[[Autoimmune hepatitis]]. | |||
*Drugs. | |||
*[[Hodgkin's lymphoma]].<ref name=pmid19131796>Vanishing bile duct syndrome and Hodgkin disease: a case series and review of the literature. Pass AK, McLin VA, Rushton JR, Kearney DL, Hastings CA, Margolin JF. J Pediatr Hematol Oncol. 2008 Dec;30(12):976-80. PMID 19131796.</ref> | |||
===Images=== | |||
<gallery> | |||
Image:Primary_biliary_cirrhosis_low_mag.jpg | PBC - low mag. (WC) | |||
Image:Primary_biliary_cirrhosis_intermed_mag.jpg | PBC - intermed. mag. (WC) | |||
</gallery> | |||
www: | |||
*[http://www.gidesigns.net/images/MC-copper-flower-garland-L.jpg Garland - wreath of flowers (gidesigns.net)]. | |||
===Staging PBC=== | |||
PBC is staged according to Ludwig:<ref>PBC. eMedicine.com. URL: [http://emedicine.medscape.com/article/171117-diagnosis http://emedicine.medscape.com/article/171117-diagnosis]. Accessed on: 22 September 2009.</ref> | |||
*Stage 1: Portal - inflammation or bile duct abnormalities. | |||
*Stage 2: Periportal - periportal fibrosis (enlargement of portal tracts) +/- inflammation. | |||
*Stage 3: Septal - septal fibrosis +/-inflammation in septa. | |||
*Stage 4: Cirrhosis - nodules of hepatocytes +/- inflammation. | |||
Notes: | |||
*There can be significant variation in staging on biopsy - due to variability of fibrosis in a PBC liver.<ref>J Clin Pathol. 1996 July; 49(7): 556-559. Available at: [http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=500569 http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=500569]. Accessed on: September 22, 2009.</ref> | |||
**"Worst area" in biopsy specimen is used to determine stage. | |||
==See also== | |||
*[[Medical liver disease]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Medical liver disease]] | |||