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评估抗磷脂综合征中的疾病活动度和损伤。

Assessing disease activity and damage in antiphospholipid syndrome.

机构信息

Rheumatology Department, University of Sao Paulo, Sao Paulo, Sao Paulo, Brazil.

First Department of Propaedeutic Internal Medicine, Medical School, National and Kapodistrian University of Athens, Athens, Greece.

出版信息

Clin Immunol. 2023 Oct;255:109727. doi: 10.1016/j.clim.2023.109727. Epub 2023 Aug 8.

Abstract

Antiphospholipid syndrome (APS) has been characterized by a variety of vascular and pregnancy manifestations related to an interplay between thrombotic and inflammatory mechanisms, a progressive accrual of irreversible organ damage and increased morbidity and mortality rates, supporting a high need of optimal treatment approach. The lack of standardized outcome measures is a significant barrier in the design of clinical studies in APS. Disease activity (in principle reversible) and its distinction from disease damage (in principle irreversible) needs to be evaluated by validated scores for use in clinical trials but also in daily clinical practice in APS. A disease damage score in APS, the DIAPS score, has been developed and validated in external cohorts. The development of a disease activity score that will provide an accurate and reproducible rating of each disease domain, can help clinicians and researchers to comprehensively assess the activity of disease and the response to treatment, in an attempt to prevent future damage.

摘要

抗磷脂综合征 (APS) 的特征是多种血管和妊娠表现,与血栓形成和炎症机制之间的相互作用、不可逆转的器官损伤的逐渐累积以及发病率和死亡率的增加有关,这支持了对最佳治疗方法的高度需求。缺乏标准化的结局衡量指标是 APS 临床研究设计中的一个重大障碍。疾病活动度(原则上是可逆的)及其与疾病损伤(原则上是不可逆的)的区别需要通过经过验证的评分来评估,以便用于临床试验,也用于 APS 的日常临床实践。APS 中的一种疾病损伤评分,即 DIAPS 评分,已经在外部队列中得到了开发和验证。开发一种疾病活动评分,可以准确和可重复地对每个疾病域进行评分,这有助于临床医生和研究人员全面评估疾病的活动度和对治疗的反应,以试图预防未来的损伤。

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