タニアイ　マキコ   Taniai Makiko   谷合　麻紀子    所属   医学部 医学科（東京女子医科大学病院）    職種   准教授
論文種別	症例報告
言語種別	英語
査読の有無	査読なし
表題	The utility of liver transplantation to treat acute liver failure caused by adult-onset Still's disease: case reports.
掲載誌名	正式名：Clinical journal of gastroenterology 略　 称：Clin J Gastroenterol ISSNコード：18657265/18657265
掲載区分	国内
巻・号・頁	14(3),pp.866-875
著者・共著者	OGASAWARA Yuri, KOGISO Tomomi, KOTERA Yoshihito, OMORI Akiko, KONDA Naoko, SUGANO Eri, SAGAWA Takaomi, TANIAI Makiko, HARIGAI MASAYOSHI, YAMAMOTO Masakazu, TOKUSHIGE Katsutoshi
発行年月	2021/06
概要	PURPOSE: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.RESULTS: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.CONCLUSION:In AOSD cases, ALF is rarely complicated; urgent LT should be considered only for patients with AOSD-related severe ALF.
DOI	10.1007/s12328-021-01398-3
PMID	33797038