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==Diagnostic Criteria==
==Diagnostic Criteria==
The diagnosis of primary biliary cirrhosis is made in the absence of extrahepatic biliary obstruction, no other comorbid condition affecting the liver with the presence of atleast two of the following:   
The diagnosis of primary biliary cirrhosis is made in the absence of extrahepatic biliary obstruction, no other comorbid condition affecting the liver with the presence of at least two of the following:<ref name="pmid19554543">{{cite journal| author=Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ et al.| title=Primary biliary cirrhosis. | journal=Hepatology | year= 2009 | volume= 50 | issue= 1 | pages= 291-308 | pmid=19554543 | doi=10.1002/hep.22906 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19554543 }} </ref>
*An alkaline phosphatase 1.5 times the upper limit of normal
*An alkaline phosphatase 1.5 times the upper limit of normal
*Anti mitochondrial antibodies with titre 1:40 or higher
*Anti-mitochondrial antibodies with titer 1:40 or higher
*Histology of liver demonstrating primary biliary cirrhosis
*Histology of liver demonstrating primary biliary cirrhosis



Revision as of 17:26, 6 February 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aysha Anwar, M.B.B.S[2]

Overview

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

OR

Diagnostic Criteria

The diagnosis of primary biliary cirrhosis is made in the absence of extrahepatic biliary obstruction, no other comorbid condition affecting the liver with the presence of at least two of the following:[1]

  • An alkaline phosphatase 1.5 times the upper limit of normal
  • Anti-mitochondrial antibodies with titer 1:40 or higher
  • Histology of liver demonstrating primary biliary cirrhosis

Diagnostic Algorithm

Algorithm showing Diagnostic approach for primary biliary cirrhosis[2]


 
 
 
 
 
 
 
 
Abnormal ALP
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Confirm if the ALP is of liver origin
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Abnormal GGT and/or total bilirubin and/or total cholesterol
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Drug history
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
 
 
 
 
 
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Discontiue drug and observe
 
 
 
 
 
 
 
Ultrasound
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal bile ducts
 
 
Dilated bile duct(s)
 
 
Pattern suggestive of infiltration
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
AMA postitve
 
AMA negative
 
Focal lesions
 
 
Biopsy*
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Probable PBC
 
 
 
 
No
 
Yes
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Confirmation of disease and histological stage
 
 
 
 
 
 
 
 
 
CT and/or MRI
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Biopsy
 
 
 
 
 
 
 
IHBD stone
 
Liver neoplasm
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Ocassional AIH
 
Definite PBC
 
MRCP
 
 
 
Biopsy
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal
 
Strictures
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Biopsy
 
PSC
SSC
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
PBC
 
Cholestasis
Small duct PSC
VBDS
 
*Infiltration into liver
e.g.: Malignant lymphoma
 
 
 


ABBREVIATIONS: AIH - autoimmune hepatitis; ALP; alkaline phosphatase; AMA - antimitochondrial antibody; CT - Computed tomography; GGT - gamma-glutamyl transpeptidase; IHBD - intrahepatic bile duct; MRCP - magnetic resonance cholangiopancreatography; MRI - magnetic resonance imaging; PBC - primary biliary cirrhosis; PSC - primary sclerosing cholangitis; SSC - secondary sclerosing cholangitis; VBDS - vanishing bile duct syndrome

References

  1. Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ; et al. (2009). "Primary biliary cirrhosis". Hepatology. 50 (1): 291–308. doi:10.1002/hep.22906. PMID 19554543.
  2. Kumagi T, Heathcote EJ (2008). "Primary biliary cirrhosis". Orphanet J Rare Dis. 3: 1. doi:10.1186/1750-1172-3-1. PMC 2266722. PMID 18215315.

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