Autoimmune hepatitis and adult-onset Still's disease: can they coexist?

Abstract

Dear Editor, We read the article by Xia et al. [1] “An unusual case of autoimmune hepatitis in a patient with adult-onset Still's disease” with great interest. We disagree with their diagnosis of autoimmune hepatitis (AIH) in a patient with adult-onset Still's disease (AOSD). Liver dysfunction is common in AOSD, and is included as one of the minor items in the criteria for AOSD proposed by Yamaguchi et al. [2]. It can occasionally be severe and even fatal, requiring liver transplantation [3–6]. We have experienced two cases of severe hepatic failure associated with AOSD [7]. As with the patient described by Xia et al., total bilirubin increased rapidly despite high-dose glucocorticoids. Their liver dysfunction showed dramatic improvement with cyclosporine therapy [7]. A similar patient with acute hepatitis who required additional cyclosporine has also been reported [8]. The diagnosis made by Xia et al. was based on the 1999 diagnostic criteria for AIH, which employs a scoring system that has several components [9]. According to that system, they made a clinical diagnosis, but the weak point is a lack of liver histology. They emphasized the presence of hypergammaglobulinemia (26.1 g/L) and positive antinuclear antibody (ANA) (1:80). However, hypergammaglobulinemia is also common in AOSD [10]. Although negative ANA is one of the minor criteria for AOSD [2], ANA is actually positive in about 7–10% of AOSD patients [2, 11]. Thus, the possibility that liver dysfunction arose from AOSD itself cannot be ruled out. AIH is often associated with other autoimmune diseases, but AOSD is not considered to be an autoimmune disease. If the diagnosis of AOSD is evident, it is natural to consider that the liver dysfunction was due to AOSD per se, rather than to overlapping AIH. Whether the diagnosis of AIH associated with AOSD can be accepted or not requires further discussion.