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Difference between revisions of "Medical liver disease"
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→Primary biliary cirrhosis
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*Abbreviated PBC. | *Abbreviated PBC. | ||
===Epidemiology=== | ===General=== | ||
====Epidemiology==== | |||
*Female>male (~9:1).<ref>{{Ref DCHH|162}}</ref> | *Female>male (~9:1).<ref>{{Ref DCHH|162}}</ref> | ||
*Usually middle age. | *Usually middle age. | ||
*Associated with other autoimmune conditions (Sjogren's syndrome, progressive systemic sclerosis, celiac). | *Associated with other autoimmune conditions (Sjogren's syndrome, progressive systemic sclerosis, celiac). | ||
===Etiology=== | ====Etiology==== | ||
*Autoimmune. | *Autoimmune. | ||
===Serology=== | ====Serology==== | ||
*AMA+. | *AMA+. | ||
===Classic presentation=== | ====Classic presentation==== | ||
*Pruritis. | *Pruritis. | ||
===Pathophysiology=== | ====Pathophysiology==== | ||
*Septal bile duct attacked. | *Septal bile duct attacked. | ||
=== | ====Treatment==== | ||
*Ursodeoxycholic acid. | |||
*May be indication for transplant. | |||
===Microscopic=== | |||
Features: | |||
*Intraepithelial lymphocytes - in bile duct '''key feature'''. | *Intraepithelial lymphocytes - in bile duct '''key feature'''. | ||
*Bile duct epithelial cells with eosinophilic cytoplasm.<ref>OA. 11 September 2009.</ref> | *Bile duct epithelial cells with eosinophilic cytoplasm.<ref>OA. 11 September 2009.</ref> | ||
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*PSC, viral hepatitis, AIH, 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> | *PSC, viral hepatitis, AIH, 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> | ||
===Staging=== | Notes: | ||
*[[PAS stain]] useful for examining basement membrane... which is lost in PBC. | |||
*Lobular inflammation should be minimal. | |||
====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> | 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 1: Portal - inflammation or bile duct abnormalities. | ||
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*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> | *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. | **"Worst area" in biopsy specimen is used to determine stage. | ||
==Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome (AIH-PBC OS)== | ==Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome (AIH-PBC OS)== | ||