21st April 2024

Features

The classic presentation is itching and fatigue in a middle-aged female. Other features may include right upper quadrant pain, xanthelasma, clubbing, hepatosplenomegaly, and clinical signs of liver cirrhosis if advanced disease. There is a high association with other autoimmune conditions such as Sjogren’s, rheumatoid arthritis, systemic sclerosis, and thyroid disease.