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日本患者泛发性脓疱型银屑病的治疗现状:一项回顾性病历审查

Treatment Status for Generalized Pustular Psoriasis in Japanese Patients: A Retrospective Chart Review.

作者信息

Okubo Yukari, Okuyama Ryuhei, Imafuku Shinichi, Tada Yayoi, Yamanaka Keiichi, Sugiura Kazumitsu, Yamaguchi Yukie, Yasuda Masahito, Sakamoto Wataru, Saitoh Morihisa, Morita Akimichi

机构信息

Department of Dermatology, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.

Department of Dermatology, School of Medicine, Shinshu University, Asahi 3-1-1, Matsumoto, 390-8621, Japan.

出版信息

Dermatol Ther (Heidelb). 2025 Jul;15(7):1883-1899. doi: 10.1007/s13555-025-01429-8. Epub 2025 May 19.

Abstract

INTRODUCTION

Generalized pustular psoriasis (GPP) is a rare, severe, and chronic inflammatory skin disease characterized by widespread pustules that leads to unpredictable and potentially serious disease flares. Information regarding treatment status for GPP and treatment patterns for flares is important but limited as a result of the rarity of the condition. We conducted a 10-year, retrospective, longitudinal chart review of treatment patterns at GPP referral hospitals in Japan.

METHODS

Eligible patients with GPP had at least 6 months of continuous observation data within 10 years after the date of protocol approval. Data were collected from patient records and annual patient reports. Patient characteristics and treatment details, including in relation to flare occurrence, were analyzed.

RESULTS

The median age of patients (N = 205) was 53 years; 48.3% were female and most had mild or moderate GPP (66.8%). Patients commonly received nonbiologic systemic therapy (86.3%) and a similar proportion received biologics (79.5%); 95.1% received topical treatment and 22.4% received systemic adrenal corticosteroids. Use of nonbiologic systemic therapy decreased, and use of biologics increased, over the study period. During the observation period, the proportion of patients receiving biologic therapy increased after a flare (from 41.4% receiving biologics when flares occurred to 62.9% initiating a new biologic post flare).

CONCLUSION

In Japanese clinical practice, the evolution of treatment practices for GPP has seen an increased use of biologic therapies over time. Biologic use was common after flares; however, some flares occurred during biologic therapy, indicating a need for improved treatment options to maintain stable disease and prevent flares.

摘要

引言

泛发性脓疱型银屑病(GPP)是一种罕见、严重的慢性炎症性皮肤病,其特征为广泛的脓疱,可导致不可预测且可能严重的疾病发作。由于该病罕见,关于GPP的治疗现状及发作期治疗模式的信息虽很重要但却有限。我们对日本GPP转诊医院的治疗模式进行了为期10年的回顾性纵向病历审查。

方法

符合条件的GPP患者在方案批准日期后的10年内至少有6个月的连续观察数据。数据从患者记录和年度患者报告中收集。分析了患者特征和治疗细节,包括与发作相关的情况。

结果

患者(N = 205)的中位年龄为53岁;48.3%为女性,大多数患有轻度或中度GPP(66.8%)。患者普遍接受非生物系统性治疗(86.3%),接受生物制剂治疗的比例相近(79.5%);95.1%接受局部治疗,22.4%接受全身性肾上腺皮质类固醇治疗。在研究期间,非生物系统性治疗的使用减少,而生物制剂的使用增加。在观察期内,发作后接受生物治疗的患者比例增加(从发作时接受生物制剂治疗者的41.4%增至发作后开始使用新生物制剂者的62.9%)。

结论

在日本的临床实践中,随着时间的推移,GPP的治疗方式演变呈现出生物治疗使用增加的趋势。发作后生物制剂的使用很常见;然而,一些发作发生在生物治疗期间,这表明需要改进治疗方案以维持疾病稳定并预防发作。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ac9/12126438/178727a528dd/13555_2025_1429_Fig1_HTML.jpg

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