大疱性类天疱疮患者的死亡率和预后因素：一项回顾性多中心意大利研究。
Mortality and prognostic factors in patients with bullous pemphigoid: a retrospective multicenter Italian study.
机构信息
Division of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, Division of Dermatology, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
Division of Dermatology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
出版信息
J Eur Acad Dermatol Venereol. 2022 Dec;36(12):2473-2481. doi: 10.1111/jdv.18441. Epub 2022 Sep 10.
INTRODUCTION
Bullous pemphigoid is the most common autoimmune bullous dermatosis. In recent years several studies have tried to identify the main factors of the disease related with an increased risk of death. The aim of this multicenter Italian study was to assess the risk score of death considering epidemiologic, clinical, immunological, and therapeutic factors in a cohort of patients affected by bullous pemphigoid and try to identify the cumulative survival up to 120 months.
METHODS
We retrospectively reviewed the medical records of patients with bullous pemphigoid who were diagnosed between 2005 and 2020 in the 12 Italian centers. Data collected included sex, age at the time of diagnosis, laboratory findings, severity of disease, time at death/censoring, treatment, and multimorbidity.
RESULTS
A total of 572 patients were included in the study. The crude mortality rate was 20.6%, with an incidence mortality rate of 5.9 × 100 person/year. The mortality rate at 1, 3, 5, and 10 years was 3.2%, 18.2%, 27.4% and 51.9%, respectively. Multivariate model results showed that the risk of death was significantly higher in patients older than 78 years, in presence of multimorbidity, anti-BP180 autoantibodies >72 U/mL, or anti-BP230 > 3 U/mL at diagnosis. The variables jointly included provided an accuracy (Harrel's Index) of 77% for predicting mortality.
CONCLUSION
This study represents the first nationwide Italian study to have retrospectively investigated the mortality rates and prognostic factors in patients with bullous pemphigoid. A novel finding emerged in our study is that a risk prediction rule based on simple risk factors (age, multimorbidity, steroid-sparing drugs, prednisone use, and disease severity) jointly considered with two biomarkers routinely measured in clinical practice (anti-BP230 and anti-BP180 autoantibodies) provided about 80% accuracy for predicting mortality in large series of patients with this disease.
简介
大疱性类天疱疮是最常见的自身免疫性大疱性皮肤病。近年来，多项研究试图确定与死亡风险增加相关的疾病主要因素。本项多中心意大利研究的目的是评估在大疱性类天疱疮患者队列中，考虑到流行病学、临床、免疫学和治疗因素的死亡风险评分，并尝试确定 120 个月的累积生存率。
方法
我们回顾性分析了 2005 年至 2020 年间在意大利 12 个中心诊断为大疱性类天疱疮的患者的病历。收集的数据包括性别、诊断时的年龄、实验室结果、疾病严重程度、死亡/删失时间、治疗和合并症。
结果
共有 572 例患者纳入本研究。粗死亡率为 20.6%，发病率死亡率为 5.9×100 人/年。1、3、5 和 10 年的死亡率分别为 3.2%、18.2%、27.4%和 51.9%。多变量模型结果显示，年龄大于 78 岁、合并症、诊断时抗 BP180 自身抗体>72U/mL 或抗 BP230>3U/mL 的患者死亡风险显著更高。共同纳入的变量为死亡率预测提供了 77%的准确性（哈雷尔指数）。
结论
本研究是第一项回顾性调查大疱性类天疱疮患者死亡率和预后因素的意大利全国性研究。本研究的一个新发现是，基于简单危险因素（年龄、合并症、皮质类固醇保留药物、泼尼松使用和疾病严重程度）联合两个在临床实践中常规测量的生物标志物（抗 BP230 和抗 BP180 自身抗体）的风险预测规则，可对该疾病大系列患者的死亡率进行约 80%的准确预测。
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