Difference between revisions of "Primary biliary cholangitis"

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#redirect [[Medical_liver_disease#Primary_biliary_cirrhosis]]
'''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]]

Revision as of 22:06, 11 November 2013

Primary biliary cirrhosis, abbreviated PBC, is a rare medical liver disease.

General

Epidemiology:

Etiology:

  • Autoimmune.

Serology:

Classic presentation:

  • Pruritis.

Pathophysiology:

  • Septal bile duct attacked.

Treatment:

  • Ursodeoxycholic acid.
  • May be indication for transplant.

Microscopic

Features:

  • "Florid duct lesion":[3]
    • Intraepithelial lymphocytes - in bile duct - key feature.
    • Bile duct epithelial cells with eosinophilic cytoplasm.[4]
  • 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).[5]

Notes:

DDx:[7]

Images

  • PBC - low mag. (WC)

  • PBC - intermed. mag. (WC)

www:

Staging PBC

PBC is staged according to Ludwig:[9]

  • 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.[10]
    • "Worst area" in biopsy specimen is used to determine stage.

See also

References

  1. ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 162. ISBN 978-0470519035.
  2. ↑ Nguyen, DL.; Juran, BD.; Lazaridis, KN. (Oct 2010). "Primary biliary cirrhosis.". Best Pract Res Clin Gastroenterol 24 (5): 647-54. doi:10.1016/j.bpg.2010.07.006. PMID 20955967.
  3. ↑ Nakanuma, Y.; Harada, K. (Sep 1993). "Florid duct lesion in primary biliary cirrhosis shows highly proliferative activities.". J Hepatol 19 (2): 216-21. PMID 7905494.
  4. ↑ OA. 11 September 2009.
  5. ↑ http://dictionary.reference.com/browse/garland
  6. ↑ Grimm, D.; Thimme, R. (Apr 2011). "[Cholestatic liver diseases].". Ther Umsch 68 (4): 195-9. doi:10.1024/0040-5930/a000150. PMID 21452140.
  7. ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 163. ISBN 978-0470519035.
  8. ↑ 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.
  9. ↑ PBC. eMedicine.com. URL: http://emedicine.medscape.com/article/171117-diagnosis. Accessed on: 22 September 2009.
  10. ↑ J Clin Pathol. 1996 July; 49(7): 556-559. Available at: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=500569. Accessed on: September 22, 2009.
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