Primary biliary cirrhosis diagnostic study of choice

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

Overview

Anti-mitochondrial antibody (AMA) titer is the diagnostic study of choice for the diagnosis of primary biliary cirrhosis. 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 criteria including an alkaline phosphatase 1.5 times the upper limit of normal, anti-mitochondrial antibodies with titer 1:40 or higher, and histology of liver demonstrating primary biliary cirrhosis.

Diagnostic Study of Choice

  • Anti-mitochondrial antibody (AMA) titer is the diagnostic study of choice for the diagnosis of primary biliary cirrhosis.[1][2]
  • A titer of > 1:40 is suggestive of primary biliary cirrhosis.
Test Sensitivity Specificity
AMA titer 95% 98%

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:[3]

Diagnostic Algorithm

Algorithm showing Diagnostic approach for primary biliary cirrhosis[4]


 
 
 
 
 
 
 
 
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. Muratori L, Granito A, Muratori P, Pappas G, Bianchi FB (2008). "Antimitochondrial antibodies and other antibodies in primary biliary cirrhosis: diagnostic and prognostic value". Clin Liver Dis. 12 (2): 261–76, vii. doi:10.1016/j.cld.2008.02.009. PMID 18456179.
  2. WALKER JG, DONIACH D, ROITT IM, SHERLOCK S (1965). "SEROLOGICAL TESTS IN DIAGNOSIS OF PRIMARY BILIARY CIRRHOSIS". Lancet. 1 (7390): 827–31. PMID 14263538.
  3. 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.
  4. 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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