Adult-onset Still's disease with secondary haemophagocytic lymphohistiocytosis induced acute liver failure: A case series

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder that very rarely causes acute liver failure (ALF), with few documented cases in the literature requiring liver transplantation. Haemophagocytic lymphohistiocytosis (HLH) is an underrecognised life-threatening hyperinflammatory syndrome that can develop in adults secondary to autoimmune conditions, post organ transplantation or in the context of infection or malignancy. We present the experience of a single tertiary liver transplant centre, with cases of two patients who developed ALF on the background of AOSD. Both cases developed secondary HLH, were successfully treated with anakinra and received super-urgent liver transplantation with good outcomes. This series highlights a rare cause of ALF and that liver transplantation can be lifesaving, although post transplantation recovery is likely to be slow and challenging.