Autoimmune cholangitis presented as fever of unknown origin.

Autoimmune cholangitis is a rare chronic cholestatic liver disease. Fever of unknown origin is defined as a temperature higher than 38.3 degrees C that lasts for more than 3 weeks with no obvious source despite appropriate investigation. We describe the case of a 62-year-old woman who presented with fever, fatigue and weight loss. The serum biochemical study showed an increase in alkaline phosphatase and gamma-glutamyl transpeptidase levels. Antinuclear, antimitochondrial, anti-smooth-muscle antibodies and antibodies against the cytoplasm of neutrophils were negative. Liver biopsy was compatible with autoimmune cholangitis. The patient was successfully treated with methylprednisolone and ursodeoxycholic acid. We describe here a rare case of fever as preceding and leading symptom of autoimmune cholangitis.