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: 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 atleast two of the following:

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

Diagnostic Algorithm

Algorithm showing Diagnostic approach for primary biliary cirrhosis

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