GPP 是一种罕见的、复发性或持续发作的严重皮肤疾病^{[1],[2]<\/sup>，临床上与斑块状银屑病不同^{[4],[5]<\/sup>。GPP是由中性粒细胞（一种白细胞）在皮肤中积聚引起的，会在全身广泛爆发疼痛性的无菌性脓疱^{[4],[5]<\/sup>。GPP患者的临床病程各不相同，有些患者可反复发作，而另一些患者则表现为疾病持续存在且间歇性发作^{[4]<\/sup>。虽然GPP发作的严重程度各不相同，但若不及时治疗，可能会出现败血症和多系统器官衰竭等并发症，可能会危及生命^{[5]<\/sup>。这种慢性全身性疾病会对患者的生活质量产生重大影响，并增加医疗负担^{[11]<\/sup>。GPP在不同区域的患病率各不相同，女性患者多于男性患者^{[5],[11],[12],[13],[14]<\/sup>。对于能够快速解决GPP发作症状并能减少复发，且具有可接受的安全性的疗法存在着高度未满足的需求^{[2],[6]<\/sup>。GPP的发作可导致住院并可能发生严重并发症，包括心力衰竭、肾衰竭和败血症，而疾病发作的不可预测性和严重程度极大地影响了患者的生活质量^{[5],[6]<\/sup>。<\/p> \n}}}}}}}}}

圣利卓^{®<\/sup><\/b>家族（通用名：佩索利单抗）包含静脉注射制剂和皮下注射制剂，是一款新型人源化选择性抗体，可阻断白介素-36受体 (IL-36R)的激活。IL-36通路是免疫系统内的一种信号通路，已被证明与GPP等多种自身炎症性疾病的发病机制有关^{[5],[7],[8]<\/sup>。佩索利单抗静脉注射制剂已获得包括美国、日本、中国和欧盟等多国监管机构批准，用于治疗成人GPP发作。<\/p> \n}}

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[1] Lebwohl M et al. Spesolimab for the prevention of generalized pustular psoriasis (GPP) flares: Results from the randomized, placebo-controlled trial Effisayil 2. Abstract presented at the 25th World Congress of Dermatology July 2023, Singapore.<\/span><\/p> <\/td> \n <\/tr> \n
[2] 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
[3] Bachelez, H. Pustular psoriasis: the dawn of a new era. Acta Derm Venereol. 2020;100(3):adv000343<\/span><\/p> <\/td> \n <\/tr> \n
[4] 《我国泛发性脓疱型银屑病的患病率和疾病负担调查：一项基于全国2012-2016年城镇医疗保险数据的估算》，中华医学会第二十七次全国皮肤性病学学术年会，2021年6月<\/span><\/p> <\/td> \n <\/tr> \n
[5] Navarini AA, et al. European consensus statement on phenotypes of pustular psoriasis. JEADV. 2017;31:1792-1799.<\/span><\/p> <\/td> \n <\/tr> \n
[6] 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
[7] Puig L, Choon SE, Gottlieb AB, et al. Generalized pustular psoriasis: a global Delphi consensus on clinical course, diagnosis, treatment goals, and disease management. J Eur Acad Dermatol Venereol. 2023 Jan 6. doi: 10.1111\/jdv.18851.<\/span><\/p> <\/td> \n <\/tr> \n
[8] 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
[9] Bachelez H, et al. Inhibition of the Interleukin-36 Pathway for the Treatment of Generalized Pustular Psoriasis. N Engl J Med. 2019; 380:981-983.<\/span><\/p> <\/td> \n <\/tr> \n
[10] Lebwohl M et al. Spesolimab for the prevention of generalized pustular psoriasis (GPP) flares: Results from the randomized, placebo-controlled trial Effisayil 2. Abstract presented at the 25th World Congress of Dermatology July 2023, Singapore.<\/span><\/p> <\/td> \n <\/tr> \n
[11] Hanna M, et al. Economic burden of generalized pustular psoriasis and palmoplantar pustulosis in the United States. Curr Med Res Opin. 2021. 37(5):735-742.<\/span><\/p> <\/td> \n <\/tr> \n
[12] Augey F, et al. Generalized pustular psoriasis (Zumbusch): a French epidemiological survey. Eur J Derm. 2006; 16(6):669-673.<\/span><\/p> <\/td> \n <\/tr> \n
[13] Ohkawara A et al. Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background. Acta Derm Venereol. 1996 Jan;76(1):68–71.<\/span><\/p> <\/td> \n <\/tr> \n
[14] Jin H, et al. Clinical features and course of generalized pustular psoriasis in Korea. J Dermatol. 2015; 42(7):674-678.<\/span><\/p> <\/td> \n <\/tr> \n <\/tbody> \n <\/table> \n<\/div> \n
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