Primary Biliary Cirrhosis

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Step 1: View clinicals

A 45 year old woman presents with jaundice and pruritus for 2 weeks. She is otherwise asymptomatic. She had a similar episode 1 year ago, which lasted for a few weeks and resolved spontaneously. She did not seek medical advice, and was symptom free afterwards. She is not on any medications, only drinks socially, and denies promiscuity or drug abuse. A full blood count is within normal parameters. An ultrasound scan of the abdomen reveals mild hepatomegaly only, with no evidence of gallstones.


Step 2: Order all relevant investigations

Liver Function Tests

AST: 90 U/L (<35) ALT: 123 U/L (<35) ALP: 972 U/L (40-125) GGT: 796 U/L (5-55) Total Serum Bilirubin: 6.1 mg/dL (<1) Direct Bilirubin: 4.2 mg/dl (0.1-0.4) INR: 2 (0.9-1.1) Albumin: 3.4 g/dL (3.4-5.4)

MRCP & MRI Abdomen

Mild hepatomegaly is present. The anatomy of the biliary tract is within normal parameters, with no evidence of obstruction or narrowing.

ANA, SMA, AMA antibodies

Antinuclear Antibodies (ANA): negative Smooth Muscle Antibodies (SMA): negative Antimitochondrial Antibody (AMA): positive

Hepatitis Virus Panel

HBsAg: Negative Anti-HBs: Negative HBeAg: Negative Anti-HBe: Negative Anti-HBc: Negative HBV DNA: Negative HCV RNA: Negative Anti HDV total: Negative


Step 3: Select appropriate management

Ursodeoxycholic Acid
Corticosteroids
Cholestyramine
Liver Transplantation


Score: ★★☆