P140 | Acute hepatitis in adult-onset Still’s disease: four presentation patterns proposed

D. Tirotta¹, C. Lena¹, P. Guasti¹, P. Muratori² | ¹Internal Medicine, Morgagni Pierantoni Unit, Forlì, AUSL Romagna, Italy, ²S Orsola University Hospital, Bologna, Italy

Background: Alteration of liver tests is generally present in adult onset Still’s disease [AOSD], but acute severe hepatitis (AH) is an unusual manifestation. Purpose of the study: We performed a sensible research of the literature on PubMed and Google scholar, investigating cases of AOSD with AH. We obtained 79 cases, of which 73 were excluded due to lack of liver biopsy or because the presence of a transient elevation of liver enzymes. We divided the spectrum of clinical manifestation of AH associated to AOSD into 4 main categories: autoimmune hepatitis, drug induced hepatitis [DILI], AH due to AOSD, haemophagocitic lymphohistiocytosis syndrome.
Results: Six cases were collected including our case: -DILI: hepatotoxicity from anakinra is transient and rare, toxicity from ciclosporin, suspected in our case, is very rare. DILI is described due to corticosteroids and tolicizumab. -Infectious etiology: CMV infection is frequent in elderly onset patients, but decreased with early corticosteroid dose tapering and immunosuppressant increased. -Involvement of the reticuloendothelial system in hemophagocytic syndrome: usually pancytopenia, hypertriglycemia are described, HS score is compatible. -Autoimmune etiology: rare, but onset under steroids is described.
Conclusions: Although some cases worsened until liver transplantation, clinical evolution of AH in AOSD is often favourable, despite persistent symptoms, but for a good prognosis an early diagnosis is important. We think that the subdivision into 4 histopathological patterns of AH in AOSD could be very helpful for patient management.