Liver involvement in adult-onset Still's disease: our experience in a third level liver unit and review of the literature

Adult-onset Still's Disease (AOSD) is a systemic inflammatory condition, mainly characterized by high spiking fevers, leukocytosis, skin rash, arthralgia and myalgia. Liver involvement is a frequent feature, usually presenting with hepatomegaly and mild liver enzymes abnormalities, which usually normalize after treatment with anti-inflammatory or immunomodulatory drugs given for AOSD. Although uncommon, the onset of severe acute hepatitis and even of life-threatening liver failure is possible and requires a prompt diagnosis and an aggressive therapy and, in some cases, an emergency liver transplantation. The differential diagnosis of the cause of the liver injury can be very challenging in these patients. We reviewed the charts of all consecutive patients admitted for acute hepatitis, between January 2019 and December 2019, to the unit of Gastroenterology and Hepatology, Molinette Hospital, Turin, Italy, searching for episodes AOSD-related. In this period, 21 cases of acute hepatitis were recorded with one among them diagnosed as due to AOSD. The incidence was 5% (1/21). This patient was a woman with a recent diagnosis of AOSD who developed a severe acute seronegative biopsy-proven autoimmune hepatitis. She was successfully treated with high-dose methylprednisolone, with a full and stable recovery from the liver injury. We discussED the incidence, etiology, pathophysiology, diagnosis, and standard of treatment in the clinical management of AOSD with a special attention and a systematic review on the available therapies for severe liver involvement associated with AOSD.