Macrophage Activation Syndrome Associated with Adult-onset Still's Disease Successfully Treated with Plasma Exchange and Canakinumab
Abstract
We herein describe the case of a 20-year-old Japanese woman with adult-onset Still's disease (AOSD) complicated by macrophage activation syndrome (MAS). Despite the administration of prednisolone and tocilizumab, MAS developed with hyperferritinemia, thrombocytopenia, hypertriglyceridemia, and liver dysfunction. Steroid pulse therapy, cyclosporine A, and plasma exchange (PE) improved the patient's condition. However, diabetes hinders glycemic control under high-dose steroids. Subcutaneous canakinumab (300 mg/month) was initiated, thus enabling rapid prednisolone tapering to 10 mg within one month without relapse. This case highlights that PE followed by canakinumab is a potentially effective strategy for treating severe AOSD with MAS, particularly when minimizing steroid exposure is critical.
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BibTeX
@article{kiyohara2026macrophage,
title = {{Macrophage Activation Syndrome Associated with Adult-onset Still's Disease Successfully Treated with Plasma Exchange and Canakinumab}},
author = {Wataru Kiyohara and Yuya Fujita and Muneyuki Hatta and Takashi Nakazawa and Shunichi Kinoshita and Yoshinori Kakutani and Kenya Sugimoto and Yukina Yokoyama and Ryuhei Ishihara and Masao Katsushima and Kazuo Fukumoto and Ryu Watanabe and Shinsuke Yamada and Masanori Emoto and Motomu Hashimoto},
journal = {Internal Medicine},
year = {2026},
month = sep,
doi = {10.2169/internalmedicine.6642-25},
url = {https://www.jstage.jst.go.jp/article/internalmedicine/65/17/65_6642-25/_article},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Macrophage Activation Syndrome Associated with Adult-onset Still's Disease Successfully Treated with Plasma Exchange and Canakinumab
AU - Wataru Kiyohara
AU - Yuya Fujita
AU - Muneyuki Hatta
AU - Takashi Nakazawa
AU - Shunichi Kinoshita
AU - Yoshinori Kakutani
AU - Kenya Sugimoto
AU - Yukina Yokoyama
AU - Ryuhei Ishihara
AU - Masao Katsushima
AU - Kazuo Fukumoto
AU - Ryu Watanabe
AU - Shinsuke Yamada
AU - Masanori Emoto
AU - Motomu Hashimoto
PY - 2026
DA - 2026/09/01
JO - Internal Medicine
DO - 10.2169/internalmedicine.6642-25
UR - https://www.jstage.jst.go.jp/article/internalmedicine/65/17/65_6642-25/_article
AB - We herein describe the case of a 20-year-old Japanese woman with adult-onset Still's disease (AOSD) complicated by macrophage activation syndrome (MAS). Despite the administration of prednisolone and tocilizumab, MAS developed with hyperferritinemia, thrombocytopenia, hypertriglyceridemia, and liver dysfunction. Steroid pulse therapy, cyclosporine A, and plasma exchange (PE) improved the patient's condition. However, diabetes hinders glycemic control under high-dose steroids. Subcutaneous canakinumab (300 mg/month) was initiated, thus enabling rapid prednisolone tapering to 10 mg within one month without relapse. This case highlights that PE followed by canakinumab is a potentially effective strategy for treating severe AOSD with MAS, particularly when minimizing steroid exposure is critical.
SN - 1349-7235
SN - 0918-2918
ER -