Department of Internal Medicine, Institute of Gastroenterology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.
Department of Surgery, Institute of Gastroenterology, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.
Clin J Gastroenterol. 2021 Jun;14(3):866-875. doi: 10.1007/s12328-021-01398-3. Epub 2021 Apr 1.
Adult-onset Still's disease (AOSD) is an inflammatory condition commonly complicated by mild liver dysfunction. However, severe liver failure is rarely reported. We report three cases of severe acute hepatic failure (ALF) associated with AOSD. We encountered three cases of acute liver failure (ALF) with encephalopathy.
Case 1 was a 75-year-old female, who was started on a steroid (prednisolone, PSL) to treat AOSD; this was gradually tapered. Two months later, severe ALF developed. She died despite an increase in the PSL dose and artificial liver support. Case 2 was a 26-year-old-female taking PSL 30 mg/day to treat subacute thyroiditis. PSL was tapered, and she received methyl PSL pulse therapy and artificial liver support, but this did not cure the ALF. Liver transplantation (LT) was performed 25 days later. Three years later, the same symptoms were observed and we diagnosed AOSD. Case 3 was a 56-year-old-female who met the AOSD criteria. PSL 50 mg/day was started and then tapered. Methyl PSL pulse therapy was prescribed to treat hemophagocytic syndrome, but she required LT on hospital day 13.
In AOSD cases, ALF is rarely complicated; urgent LT should be considered only for patients with AOSD-related severe ALF.
成人Still 病（AOSD）是一种常见的伴有轻度肝功能障碍的炎症性疾病。然而，严重肝衰竭很少见。我们报告三例与 AOSD 相关的严重急性肝衰竭（ALF）。我们遇到了三例伴有肝性脑病的急性肝衰竭（ALF）。
病例 1 为 75 岁女性，因 AOSD 开始接受皮质类固醇（泼尼松龙，PSL）治疗；逐渐减量。两个月后，发生严重的 ALF。尽管增加了 PSL 剂量和人工肝支持，她仍死亡。病例 2 为 26 岁女性，因亚急性甲状腺炎服用 PSL 30mg/天。PSL 逐渐减量，接受甲基 PSL 脉冲治疗和人工肝支持，但未能治愈 ALF。25 天后进行了肝移植（LT）。三年后，出现相同症状，诊断为 AOSD。病例 3 为 56 岁女性，符合 AOSD 标准。开始服用 50mg/天的 PSL，然后逐渐减量。给予甲基 PSL 脉冲治疗以治疗噬血细胞综合征，但在入院第 13 天需要 LT。
在 AOSD 病例中，ALF 很少合并；仅对 AOSD 相关严重 ALF 患者考虑紧急 LT。
