Primary biliary cirrhosis differential diagnosis: Difference between revisions

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{{Primary biliary cirrhosis}}
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Primary_biliary_cirrhosis]]


{{CMG}}; {{AE}}
{{CMG}}; {{AE}} {{MJ}}, {{Anmol}}


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==Overview==
Primary biliary cirrhosis must be differentiated from other disease that may cause [[cholestasis]] including [[autoimmune hepatitis]], [[common bile duct]] stone, [[hepatitis A]] ([[choelstatic]] type), [[EBV]] or [[CMV]] [[hepatitis]], [[primary sclerosing cholangitis]], pre-ampullary cancers, [[AIDS]] cholangiopathy, [[parasites]] induced [[cholestasis]], and [[intrahepatic cholestasis of pregnancy]].


==Overview==
==Differentiating primary biliary cirrhosis from other Diseases==
Primary biliary cirrhosis must be differentiated from other disease that may cause [[cholestasis]].<ref name="pmid28145671">{{cite journal |vauthors=Fargo MV, Grogan SP, Saguil A |title=Evaluation of Jaundice in Adults |journal=Am Fam Physician |volume=95 |issue=3 |pages=164–168 |year=2017 |pmid=28145671 |doi= |url=}}</ref><ref name="pmid9287990">{{cite journal |vauthors=Leevy CB, Koneru B, Klein KM |title=Recurrent familial prolonged intrahepatic cholestasis of pregnancy associated with chronic liver disease |journal=Gastroenterology |volume=113 |issue=3 |pages=966–72 |year=1997 |pmid=9287990 |doi= |url=}}</ref><ref name="pmid18609700">{{cite journal |vauthors=Hov JR, Boberg KM, Karlsen TH |title=Autoantibodies in primary sclerosing cholangitis |journal=World J. Gastroenterol. |volume=14 |issue=24 |pages=3781–91 |year=2008 |pmid=18609700 |pmc=2721433 |doi= |url=}}</ref><ref name="pmid3115390">{{cite journal |vauthors=Bond LR, Hatty SR, Horn ME, Dick M, Meire HB, Bellingham AJ |title=Gall stones in sickle cell disease in the United Kingdom |journal=Br Med J (Clin Res Ed) |volume=295 |issue=6592 |pages=234–6 |year=1987 |pmid=3115390 |pmc=1247079 |doi= |url=}}</ref><ref name="pmid29226106">{{cite journal |vauthors=Malakouti M, Kataria A, Ali SK, Schenker S |title=Elevated Liver Enzymes in Asymptomatic Patients - What Should I Do? |journal=J Clin Transl Hepatol |volume=5 |issue=4 |pages=394–403 |year=2017 |pmid=29226106 |pmc=5719197 |doi=10.14218/JCTH.2017.00027 |url=}}</ref>


==Differential Diagnosis==
{| class="wikitable" align="center"
|-
|
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
! rowspan="3" align="center" style="background:#4479BA; color: #FFFFFF;" |Disease
! colspan="4" rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |History and clinical manifestations
! colspan="8" align="center" style="background:#4479BA; color: #FFFFFF;" |Diagnosis
|-
! colspan="6" align="center" style="background:#4479BA; color: #FFFFFF;" |Lab Findings
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Other blood tests
! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" |Other diagnostic
|-
! rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" |Family history
! align="center" style="background:#4479BA; color: #FFFFFF;" |Fever
! align="center" style="background:#4479BA; color: #FFFFFF;" |RUQ Pain
! align="center" style="background:#4479BA; color: #FFFFFF;" |Pruritis
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" |AST
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" |ALT
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" |ALP
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" |BLR Indirect
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" |BLR Direct
! colspan="1" rowspan="1" align="center" style="background:#4479BA; color: #FFFFFF;" |Viral serology
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Primary biliary cirrhosis]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |AMA positive
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Liver biopsy
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Autoimmune hepatitis]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑/N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Anti-LKM antibody]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Liver biopsy
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |Common bile duct stone
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Dilated ducts on ultrasound
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |CT/[[Endoscopic retrograde cholangiopancreatography|ERCP]]
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Hepatitis A]] (cholestatic type)
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |HAV- Ab
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Abdominal ultrasound
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Epstein Barr virus|EBV]] / [[Cytomegalovirus infection|CMV]] hepatitis
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Positive serology
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Primary sclerosing cholangitis]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑Autoantibodies (P-ANCA), [[hypergammaglobulinemia]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Magnetic resonance cholangiopancreatography|MRCP]],
Liver biopsy
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |Pre-ampullary cancers
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |CT scan for diagnosis
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[HIV AIDS|AIDS]] cholangiopathy
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |HIV Ab
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Ultrasound or [[Endoscopic retrograde cholangiopancreatography|ERCP]]
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |Parasites induces cholestasis
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N/↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Serology
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Ultrasound or [[Endoscopic retrograde cholangiopancreatography|ERCP]]
|-
| colspan="1" rowspan="1" style="padding: 5px 5px; background: #DCDCDC;" align="center" |[[Intrahepatic cholestasis of pregnancy]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -/+
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | +
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |N
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |↑
| style="padding: 5px 5px; background: #F5F5F5;" align="center" | -
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |[[Thrombocytopenia]]
| style="padding: 5px 5px; background: #F5F5F5;" align="center" |Diagnosed clinically
|-
|}
|}


==References==
==References==
{{reflist|2}}
{{Reflist|2}}
 
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[[Category:Gastroenterology]]
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[[Category:Hepatology]]
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Latest revision as of 23:49, 29 July 2020

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2], Anmol Pitliya, M.B.B.S. M.D.[3]

Overview

Primary biliary cirrhosis must be differentiated from other disease that may cause cholestasis including autoimmune hepatitis, common bile duct stone, hepatitis A (choelstatic type), EBV or CMV hepatitis, primary sclerosing cholangitis, pre-ampullary cancers, AIDS cholangiopathy, parasites induced cholestasis, and intrahepatic cholestasis of pregnancy.

Differentiating primary biliary cirrhosis from other Diseases

Primary biliary cirrhosis must be differentiated from other disease that may cause cholestasis.[1][2][3][4][5]

Disease History and clinical manifestations Diagnosis
Lab Findings Other blood tests Other diagnostic
Family history Fever RUQ Pain Pruritis AST ALT ALP BLR Indirect BLR Direct Viral serology
Primary biliary cirrhosis -/+ - -/+ + N/↑ N/↑ ↑ N ↑ - AMA positive Liver biopsy
Autoimmune hepatitis -/+ - - -/+ ↑ ↑ N ↑/N N - Anti-LKM antibody Liver biopsy
Common bile duct stone -/+ - + + N N ↑ N ↑ - Dilated ducts on ultrasound CT/ERCP
Hepatitis A (cholestatic type) - -/+ + + N/↑ N/↑ ↑ N ↑ + HAV- Ab Abdominal ultrasound
EBV / CMV hepatitis - -/+ + + N N ↑ N ↑ + Positive serology -
Primary sclerosing cholangitis -/+ - -/+ + N/↑ N/↑ ↑ N ↑ - ↑Autoantibodies (P-ANCA), hypergammaglobulinemia MRCP,

Liver biopsy

Pre-ampullary cancers + - -/+ -/+ N/↑ N/↑ ↑ N ↑ - - CT scan for diagnosis
AIDS cholangiopathy - - -/+ -/+ N/↑ N/↑ ↑ N ↑ - HIV Ab Ultrasound or ERCP
Parasites induces cholestasis - - -/+ -/+ N/↑ N/↑ ↑ N ↑ - Serology Ultrasound or ERCP
Intrahepatic cholestasis of pregnancy -/+ - -/+ + ↑ ↑ ↑ N ↑ - Thrombocytopenia Diagnosed clinically

References

  1. ↑ Fargo MV, Grogan SP, Saguil A (2017). "Evaluation of Jaundice in Adults". Am Fam Physician. 95 (3): 164–168. PMID 28145671.
  2. ↑ Leevy CB, Koneru B, Klein KM (1997). "Recurrent familial prolonged intrahepatic cholestasis of pregnancy associated with chronic liver disease". Gastroenterology. 113 (3): 966–72. PMID 9287990.
  3. ↑ Hov JR, Boberg KM, Karlsen TH (2008). "Autoantibodies in primary sclerosing cholangitis". World J. Gastroenterol. 14 (24): 3781–91. PMC 2721433. PMID 18609700.
  4. ↑ Bond LR, Hatty SR, Horn ME, Dick M, Meire HB, Bellingham AJ (1987). "Gall stones in sickle cell disease in the United Kingdom". Br Med J (Clin Res Ed). 295 (6592): 234–6. PMC 1247079. PMID 3115390.
  5. ↑ Malakouti M, Kataria A, Ali SK, Schenker S (2017). "Elevated Liver Enzymes in Asymptomatic Patients - What Should I Do?". J Clin Transl Hepatol. 5 (4): 394–403. doi:10.14218/JCTH.2017.00027. PMC 5719197. PMID 29226106.

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