High glucocorticoid dependency and limited therapeutic response in Japanese patients with VEXAS syndrome: a multicentre retrospective study.
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Maeda Ayaka, Kirino Yohei, Tsuchida Naomi, Uchiyama Yuri, Kobayashi Daisuke, Asano Tomoyuki, Ito Yuji, Katsuo Kosuke, Kato Ayaka, Mori Ichiro, Yamaguchi Hiroyuki, Shimizu Toshimasa, Kobayashi Satoshi, Kishimoto Mitsumasa, Oda Nobuhiro, Rokutanda Ryo, Nagahata Ken, Fujiwara Hiroshi, Kobayashi Hiroshi, Takeuchi Yoichi, Hagiyama Hiroyuki, Umeda Naoki, Uchino Kaori, Miyoshi Yuji, Hidaka Yukiko, Ida Hiroaki, Akasaka Keiichi, Kashino Kaori, Yoshida Yusuke, Ohnishi Hidenori, Takahashi Noriyuki, Fujieda Yuichiro, Horita Nobuyuki, Takase-Minegishi Kaoru, Matsumoto Naomichi, Nakajima Hideaki
Аннотация
METHODS: We retrospectively analysed data on clinical manifestations, treatments, and outcomes from 46 male patients diagnosed with VEXAS syndrome. Twelve patients at our institution were evaluated for remission using the French VEXAS group criteria.
RESULTS: All patients (median age at onset: 71.4 years) received systemic glucocorticoid therapy (mean maximum dose: 47.4 mg/day; mean minimum dose: 8.5 mg/day). Most patients required continuous glucocorticoid treatment of ≥10 mg/day, and tapering was generally difficult. Among the patients followed at our institution, only 42% (5/12) achieved complete remission at least once during follow-up. Tocilizumab was the most frequently administered immunosuppressant (n = 24, 52.2%); Janus kinase inhibitors and azacitidine, reported to be effective overseas, were prescribed in only three and two cases, respectively.
CONCLUSIONS: This study highlights the difficulty of treating patients with VEXAS syndrome in Japan. The development of more effective treatments is urgently needed to reduce glucocorticoid dependence and improve patient outcomes.