大疱性类天疱疮患者的临床结局和预后因素：中国15年回顾。

IF 8.6 1区医学 Q1 DERMATOLOGY

American Journal of Clinical Dermatology Pub Date : 2025-03-10 DOI:10.1007/s40257-025-00925-z

Shan Cao, Wenchao Li, Zhenzhen Wang, Hongda Li, Pengcheng Huai, Tongsheng Chu, Baoqi Yang, Yonghu Sun, Peiye Xing, Guizhi Zhou, Yongxia Liu, Shengli Chen, Qing Yang, Mei Wu, Zhongxiang Shi, Hong Liu, Furen Zhang

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引用次数: 0

摘要

背景：大疱性类天疱疮（BP）的临床结局和预后因素的长期随访数据有限。目的：探讨BP患者的临床结局及预后因素。方法：本回顾性研究于2009年1月1日至2023年12月31日在中国山东省进行。通过Cox比例风险模型或logistic回归分析，主要结局是死亡率和预测因素、完全停药缓解（CROT）和复发率。还描述了BP死亡率和CROT的nomogram。结果：纳入的1063例BP患者中，有45例因随访失败而被排除。该队列包括1018例BP患者进行分析。死亡344例（33.8%），1年、3年和5年累计死亡率分别为22.8%、31.2%和34.5%。发病年龄增加（HR = 1.08），体表面积（BSA）受累10-30%，BSA受累> 30% (HR = 7.19；HR = 9.84)、DIF双阳性IgG和C3 （HR = 1.37）以及全身皮质类固醇联合免疫抑制剂治疗（HR = 0.50）与死亡率相关。共有321例（31.5%）患者实现CROT。1年、3年和5年的累计CROT率分别为10.9%、32.9%和47.5%。诊断延迟时间较短（HR = 1.01）、基线抗bp180抗体< 50 IU/mL （HR = 1.48）和全身非皮质类固醇治疗药物（HR = 1.68）与CROT相关。预测模型在1年、3年和5年的死亡率分类方面表现出色（auc为0.83、0.86、0.88），但对CROT的分类表现一般（auc为0.67、0.62、0.63）。共有749例（73.6%）患者出现复发。结论：本研究是首个研究BP患者长期预后的大型队列研究，确定了死亡率和CROT的危险因素，为临床医生改善预后和降低复发率提供了关键见解。

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Clinical Outcomes and Prognostic Factors in Bullous Pemphigoid Patients: A 15-Year Review in China

Background

There are limited data on clinical outcomes and prognosis factors for bullous pemphigoid (BP) at long-term follow-up.

Objective

We aimed to investigate the clinical outcomes and prognostic factors in BP patients.

Methods

This retrospective study was performed between January 1, 2009 and December 31, 2023 in Shandong Province, China. The primary outcomes were the rates and predictive factors of mortality, complete remission off-therapy (CROT), and relapse by Cox proportional hazards models or logistic regression analyses. Nomograms for BP mortality and CROT were also described.

Results

Of the 1063 BP patients enrolled, 45 were excluded due to loss to follow-up. The cohort comprised 1018 BP patients to analyze. A total of 344 (33.8%) patients died, with cumulative 1-, 3-, and 5-year mortality rates of 22.8%, 31.2%, and 34.5%, respectively. Increased age at onset (HR = 1.08), body surface area (BSA) involvement 10–30%, BSA involvement > 30% (HR = 7.19; HR = 9.84, respectively), double-positive IgG and C3 on DIF (HR = 1.37), and systemic corticosteroid in combination with immunosuppressants treatments (HR = 0.50) were associated with mortality. A total of 321 (31.5%) patients achieved CROT. Cumulative CROT rates at 1, 3, and 5 years were 10.9%, 32.9%, and 47.5%, respectively. Shorter diagnosis delay time (HR = 1.01), baseline anti-BP180 antibody < 50 IU/mL (HR = 1.48) and systemic drugs other than corticosteroid treatment (HR = 1.68) were associated with CROT. Predictive models demonstrated outstanding performance in classifying mortality at 1, 3, and 5 years (AUCs 0.83, 0.86, 0.88), but moderate classification for CROT (AUCs 0.67, 0.62, 0.63). A total of 749 (73.6%) patients experienced relapses.

Conclusions

This study, the first large cohort to examine long-term outcomes in BP patients, identifies risk factors for mortality and CROT, offering key insights for clinicians to improve prognosis and reduce relapse rates.

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来源期刊

American Journal of Clinical Dermatology 医学-皮肤病学

CiteScore

15.20

自引率

2.70%

发文量

审稿时长

>12 weeks

期刊介绍： The American Journal of Clinical Dermatology is dedicated to evidence-based therapy and effective patient management in dermatology. It publishes critical review articles and clinically focused original research covering comprehensive aspects of dermatological conditions. The journal enhances visibility and educational value through features like Key Points summaries, plain language summaries, and various digital elements, ensuring accessibility and depth for a diverse readership.