Honda Daisuke, Ohsawa Isao, Mano Satoshi, Rinno Hisaki, Tomino Yasuhiko, Suzuki Yusuke
Department of Nephrology, Juntendo University Faculty of Medicine, Tokyo, Japan.
Nephrology Unit, Internal Medicine, Saiyu Soka Hospital, Saitama, Japan.
Intractable Rare Dis Res. 2021 May;10(2):142-145. doi: 10.5582/irdr.2021.01010.
Hereditary angioedema due to C1-inhibitor (C1-INH) deficiency (HAE-C1-INH) induces an acute attack of angioedema. In 2018, icatibant available for self-possession and subcutaneous self-administration was licensed for on-demand treatment in addition to intravenous C1-INH administration in Japan. We retrospectively evaluated the percentage of attacks in critical parts at emergency room (ER) visits and the time until visiting ER for C1-INH administration before and after the initial prescription of icatibant. The percentage of attacks in critical parts at ER visits before the prescription was 69.2%, but that was 80.0% when patients visited ER for additional C1-INH administration after the self-administration of icatibant. The time from the onset of an acute attack to visiting ER for the additional treatment after the self-administration of icatibant significantly increased from 6.2 h to 19.2 h ( < 0.001). Icatibant, therefore, promoted the patients' behavior modification associated with ER visits for C1-INH administration during an acute attack of HAE-C1-INH.
由于C1抑制剂（C1-INH）缺乏引起的遗传性血管性水肿（HAE-C1-INH）会引发血管性水肿急性发作。2018年，在日本，除静脉注射C1-INH外，可自行持有并皮下自行给药的依库珠单抗被批准用于按需治疗。我们回顾性评估了依库珠单抗首次处方前后，急诊室（ER）就诊时关键部位发作的百分比以及开始使用C1-INH治疗前到ER就诊的时间。处方前ER就诊时关键部位发作的百分比为69.2%，但在自行注射依库珠单抗后患者因额外使用C1-INH到ER就诊时，这一比例为80.0%。自行注射依库珠单抗后，从急性发作开始到因额外治疗到ER就诊的时间从6.2小时显著增加到19.2小时（<0.001）。因此，依库珠单抗促进了HAE-C1-INH急性发作期间患者与因使用C1-INH到ER就诊相关的行为改变。
