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A 66 year old woman presents with a 4 month history of fatigue, upper abdominal discomfort and itching. On examination, she has hepatomegaly, jaundice and hyperpigmentation. A subsequent blood test demonstrates the following:

ALT 189 U/L (normal less than 50 U/L)

AST 174 U/L (normal less than 50 U/L)

Alkaline Phosphatase 757 U/L (normal range, 30-130 U/L)

Anti-Smooth Muscle Antibody Negative

Anti-Mitochondrial Antibody Positive

Liver-Kidney Microsomal Antibody Negative


What is the most likely diagnosis?

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