Difference between revisions of "Primary biliary cholangitis"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Primary_biliary_cirrhosis_low_mag.jpg
| Width      =
| Caption    = Primary biliary cirrhosis. [[H&E stain]].
| Micro      = "Florid duct lesion": intraepithelial lymphocytes - in bile duct, bile duct epithelial cells with eosinophilic cytoplasm; plasma cells; +/-[[granulomas]] (close to bile duct); "Garland" cirrhosis -- has irregular border
| Subtypes  =
| LMDDx      = [[sarcoidosis]], [[primary sclerosing cholangitis]], [[viral hepatitis]], [[autoimmune hepatitis]], [[drug-induced liver disease]], [[Hodgkin's lymphoma]]
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[liver]] - see [[medical liver diseases]]
| Assdx      = other autoimmune conditions, e.g. [[celiac disease]], [[Sjögren syndrome]]
| Syndromes  =
| Clinicalhx = woman, middle age
| Signs      =
| Symptoms  = pruritis
| Prevalence =
| Bloodwork  = AMA +ve
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
}}
'''Primary biliary cirrhosis''', abbreviated '''PBC''', is a rare [[medical liver disease]].
'''Primary biliary cirrhosis''', abbreviated '''PBC''', is a rare [[medical liver disease]].


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*[[Sarcoidosis]] (if granulomas present).
*[[Sarcoidosis]] (if granulomas present).
*[[Primary sclerosing cholangitis]].
*[[Primary sclerosing cholangitis]].
*Viral hepatitis.
*[[Viral hepatitis]].
*[[Autoimmune hepatitis]].
*[[Autoimmune hepatitis]].
*Drugs.
*[[Drug-induced liver disease]].
*[[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>
*[[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>


Revision as of 22:12, 11 November 2013

Primary biliary cholangitis
Diagnosis in short

Primary biliary cirrhosis. H&E stain.
LM "Florid duct lesion": intraepithelial lymphocytes - in bile duct, bile duct epithelial cells with eosinophilic cytoplasm; plasma cells; +/-granulomas (close to bile duct); "Garland" cirrhosis -- has irregular border
LM DDx sarcoidosis, primary sclerosing cholangitis, viral hepatitis, autoimmune hepatitis, drug-induced liver disease, Hodgkin's lymphoma
Site liver - see medical liver diseases
Associated Dx other autoimmune conditions, e.g. celiac disease, Sjögren syndrome
Clinical history woman, middle age
Symptoms pruritis
Blood work AMA +ve

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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