Elevation of alkaline phosphatase (ALP) is reported to be an abnormal feature in TAFRO syndrome. However, the cause is unknown. Herein, we report a case of TAFRO syndrome showing intrahepatic cholangitis on autopsy, which may be the cause of ALP elevation in TAFRO syndrome.

There are no specific serological markers or imaging techniques for Adult-onset Still’s disease (AOSD) diagnosis. It is essential to rule out other diseases, especially malignant lymphoma, because both diseases sometimes show similar clinical presentations. However, unfortunately, this case showed PET-CT findings in AOSD patients could mimic those in malignant lymphoma.