亚太地区泛发性脓疱型银屑病治疗共识建议。

Siew Eng Choon, Peter Anthony Foley, Pravit Asawanonda, Hideki Fujita, Seong-Jin Jo, Yu-Ling Shi, Colin Theng, Azura Mohd Affandi, Chul Hwan Bang, Maria Lorna Frez, Huang Yu Huei, Doanh Le Huu, Tae-Gyun Kim, Akimichi Morita, Hazel H Oon, Pablo Fernández-Peñas, Natta Rajatanavin, Suganthy Robinson, Latha Selvarajah, Tsen-Fang Tsai
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引用次数: 0

摘要

泛发性脓疱型银屑病(GPP)是一种罕见的慢性、异质性疾病,有可能危及生命,其特点是原发性、无菌、大面积可见脓疱,伴有或不伴有全身症状。与 GPP 相关的基因突变存在种族差异,可能会影响临床表现和治疗反应。目前,来自亚太地区(APAC)患者群体的证据有限,导致该地区 GPP 患者的有效管理信息普遍匮乏。这项经过修改的德尔菲小组研究旨在确定当前的证据并获得先进的见解,以促进制定适合亚太地区的 GPP 管理共识。研究人员进行了系统性文献回顾 (SLR),以确定已发表的文献,并就以下方面制定共识声明:(i) GPP 的定义和临床过程;(ii) GPP 的诊断;(iii) 治疗结果、目标和监测措施;(iv) 最佳管理策略和临床实践。由皮肤科专家组成的专家小组对声明进行了两轮评分,达成共识的阈值为≥80%。来自亚太地区的 20 位专家就 106 项声明达成了共识,这些声明是根据 SLR 和专家的集体专业知识制定的。专家们一致认为,GPP 是一种罕见、严重且可能危及生命的疾病,有别于斑块状银屑病。这一共识强调了根据 GPP 病发严重程度和每位患者的独特临床情况制定针对性治疗策略的重要性。专家们就 GPP 复发的严重程度分类达成了共识,并推荐了成人 GPP、儿童 GPP 和妊娠期 GPP 的一线治疗和维持治疗方案。这些共识成果已被归纳为治疗算法,以指导亚太地区皮肤科医生的临床决策过程。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Asia-Pacific consensus recommendations on the management of generalized pustular psoriasis.

Generalized pustular psoriasis (GPP) is a rare, chronic, heterogeneous, and potentially life-threatening disease characterized by primary, sterile, and macroscopically visible pustules with or without systemic symptoms. There are ethnic differences in the genetic mutations associated with GPP that might affect the clinical manifestations and treatment responses. Currently, there is limited evidence from the patient population in the Asia-Pacific (APAC) region, resulting in a general paucity of information on the effective management of patients with GPP in this region. This modified Delphi panel study aimed to identify current evidence and gain advanced insights to facilitate the development of a regionally tailored APAC consensus on the management of GPP. A systematic literature review (SLR) was conducted to identify published literature and develop consensus statements on (i) definition and clinical course, (ii) diagnosis of GPP, (iii) treatment outcomes, goals, and monitoring measures, and (iv) optimal management strategies and clinical practices. Statements were rated by a panel of dermatologists in two rounds, with the threshold for consensus at ≥80% agreement. Twenty experts from the APAC region reached consensus on 106 statements that were developed based on the SLR and experts' collective expertise. The experts agreed that GPP is a rare, severe, and potentially life-threatening condition that is distinct from plaque psoriasis. This consensus emphasized the importance of a tailored treatment strategy taking into account the GPP flare severity and each patient's unique clinical circumstances. The experts reached consensus on the severity classification of GPP flares and recommended first-line and maintenance treatment options for adult GPP, childhood GPP, and GPP in pregnancy. These consensus outcomes have been synthesized into treatment algorithms to guide dermatologists in the APAC region in their clinical decision-making processes.

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