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
he 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 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
| 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
- ↑ 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.
- ↑ 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.