GPP 是一种罕见的、异质性的、可危及生命的严重皮肤病^{[1],[2]<\/span><\/sup>，GPP的临床表现为患者皮肤会广泛爆发脓疱，并伴有痛感，同时可能伴发高热等全身症状。治疗不及时可能导致肝肾损害，也可因继发感染、器官功能衰竭而危及生命^{[3]<\/span><\/sup>。2023年9月，GPP已被纳入《第二批罕见病目录》，这也将进一步提升GPP规范化诊疗体系的建设。<\/p> \n}}

圣利卓^{®<\/sup>（佩索利单抗注射液）是一款新型人源化选择性IgG1单克隆抗体，可阻断白介素-36受体 (IL-36R) 的激活。IL-36信号通路与GPP的发病机制密切相关，是导致皮肤炎症、脓疱形成和异常组织重构的主要细胞因子。圣利卓^{®<\/sup>通过与IL-36受体结合，从而抑制GPP的炎症信号通路，实现脓疱和皮损的快速清除^{[4],[5],[6]<\/span><\/sup>。圣利卓^{®<\/sup>用于治疗GPP发作的适应症曾获得中国国家药品监督管理局（NMPA）药品审评中心（CDE）突破性治疗药物认定。圣利卓^{®<\/sup>已于今年5月全球率先在华递交了其新适应症上市申请，用于预防GPP发作。基于其关键性EFFISAYIL™2临床试验优越的研究结果和临床价值4，CDE已授予圣利卓®该新增适应症突破性疗法认定。<\/p> \n}}}}}

间质性肺疾病（ILD）是包含200多种可导致肺纤维化疾病的总称。肺部组织的永久疤痕可致肺功能不可逆转的减退，约18%-32%的ILD患者（IPF除外）可发展为PPF，该种疾病患病人数较少，患病率仅有2.2-28.0\/100,000^{[7]<\/span><\/sup>。患病后导致肺功能下降、呼吸功能恶化、进行性肺纤维化，降低患者生命质量^{[8]<\/span><\/sup>。患者发生一次急性加重风险的比例是无\/缓慢进展ILD患者的2.7倍（19.7% vs. 7.2%），导致因急性加重的住院次数和医疗支出增加^{[9]<\/span><\/sup>，经济负担加重。。<\/p> \n}}}

维加特^{®<\/sup>是全球首个且唯一获批用于治疗具有进行性表型的慢性纤维化性间质性肺疾病的原研药品^{[10]<\/span><\/sup>，显著延缓患者用力肺活量（Forced vital capacity，FVC）年下降率达57%^{[11]<\/span><\/sup>，安全性、耐受性良好2，其作用机制明确，并获得美国食品药品监督管理局突破性疗法认定。患者用药后，与安慰剂组对比，显著降低首次急性加重或死亡风险达33%，显著降低疾病进展或死亡风险达34%^{[12]<\/span><\/sup>。<\/p> \n}}}}

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[1] Kharawala S, et al. The clinical, humanistic, and economic burden of generalized pustular psoriasis: a structured review. Exp Rev Clin Immunol. 2020;16(3):239-252.<\/span><\/p> <\/td> \n <\/tr> \n
[2] Bachelez, H. Pustular psoriasis: the dawn of a new era. Acta Derm Venereol. 2020;100(3):adv000343<\/span><\/p> <\/td> \n <\/tr> \n
[3] Crowley JJ, et al. A brief guide to pustular psoriasis for primary care providers, Postgraduate Medicine. 2021;133(3):330-344.<\/span><\/p> <\/td> \n <\/tr> \n
[4] Bachelez H et al. Trial of Spesolimab for Generalized Pustular Psoriasis. NEJM. 2021;385:2431-40<\/span><\/p> <\/td> \n <\/tr> \n
[5] Crowley JJ, et al. A brief guide to pustular psoriasis for primary care providers, Postgraduate Medicine. 2021;133(3):330-344.<\/span><\/p> <\/td> \n <\/tr> \n
[6] Furue K, et al. Highlighting Interleukin-36 Signalling in Plaque Psoriasis and Pustular Psoriasis. Acta Derm Venereol. 2018;98:5–13.<\/span><\/p> <\/td> \n <\/tr> \n
[7] Olson A, Hartmann N, Patnaik P, et al. Adv Ther. 2021 Feb;38(2):854-867.<\/span><\/p> <\/td> \n <\/tr> \n
[8] Olson A, Hartmann N, Patnaik P, et al. Adv Ther. 2021 Feb;38(2):854-867<\/span><\/p> <\/td> \n <\/tr> \n
[9] Wuyts WA, Papiris S, Manali E, et al. Adv Ther. 2020 Jul;37(7):3246-3264.<\/span><\/p> <\/td> \n <\/tr> \n
[10] https:\/\/www.fda.gov\/news-events\/press-announcements\/fda-approves-first-treatment-group-progressive-interstitial-lung-diseases<\/a><\/span><\/p> <\/td> \n <\/tr> \n
[11] Marlies Wijsenbeek, et al. Curr Med Res Opin. 2019 Nov;35(11):2015-2024.<\/span><\/p> <\/td> \n <\/tr> \n
[12] Flaherty KR et al. Eur Resp J 2020;56:4578;<\/span><\/p> <\/td> \n <\/tr> \n <\/tbody> \n <\/table> \n<\/div> \n
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