Mortality Risk Prediction in Amyopathic Dermatomyositis Associated With Interstitial Lung Disease The FLAIR Model
Mortality Risk Prediction in Amyopathic Dermatomyositis Associated With Interstitial Lung Disease The FLAIR Model
复制标题
与间质性肺病相关的无肌病性皮肌炎的死亡风险预测 FLAIR 模型
DOI：
10.1016/j.chest.2020.04.057
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发表时间：
2020-10-01
期刊：
影响因子：
9.6
通讯作者：
Bao, Chunde
中科院分区：
文献类型：
--
作者：
Lian, Xinyue;Zou, Jing;Bao, Chunde
背景：无肌病性皮肌炎（ADM）相关间质性肺病（ILD）的预后差。需要一个死亡风险评分模型来预测ADM-ILD患者的生存率，并指导临床治疗。研究问题：如何识别ADM-ILD患者的高危人群，并根据风险分层模型预测患者的预后？研究设计和方法：在这项前瞻性研究中，我们评估了207例ADM-ILD患者。我们使用多变量考克斯比例风险模型来识别独立的预后风险因素，并根据2012年1月至2016年12月的患者数据创建风险评分模型。我们使用了预测准确性指数，该指数使用Brier得分来反映模型的区分和校准。该模型在2017年1月至2018年6月的一组独立患者中进行了验证。结果：我们开发了一个综合风险评分，FLAIR评分，包括以下值和评分：铁蛋白（= 636 ng/mL，2），乳酸脱氢酶（≥ 355 U/L，2），抗黑素瘤分化相关基因5抗体（阴性，0;+，2;++，3;+，4）、高分辨率CT成像评分（= 133，3）和快速进展ILD（RPILD）（非RPILD，0; RPILD，2）。我们根据FLAIR评分将患者分为三个风险组：低，0至4;中等，5至9;和高，10至13。在发现和验证队列中，高风险患者的死亡率显著高于低风险和中风险患者（P <.001）。解释：FLAIR风险评分模型可以帮助预测ADM-ILD患者的生存率，并指导进一步的基于风险的治疗的临床研究。
BACKGROUND: The prognosis of amyopathic dermatomyositis (ADM)-associated interstitial lung disease (ILD) is poor. A mortality risk score model is needed to predict survival in patients with ADM-ILD and to guide clinical treatment.RESEARCH QUESTION: How to identify patients with ADM-ILD who are at high risk and to predict patient outcome based on a risk stratification model?STUDY DESIGN AND METHODS: We evaluated 207 patients with ADM-ILD in this prospective inception study. We used a multivariable Cox proportional hazards model to identify the independent prognostic risk factors and created a risk score model according to patient data from January 2012 to December 2016. We used the index of prediction accuracy that uses the Brier score to reflect both discrimination and calibration of the model. The model was validated in an independent group of patients from January 2017 to June 2018.RESULTS: We developed a combined risk score, the FLAIR score, that included the following values and scores: ferritin (= 636 ng/mL, 2), lactate dehydrogenase (= 355 U/L, 2), antimelanoma differentiation-associated gene 5 antibody (negative, 0; +, 2; ++, 3; +++, 4), high-resolution CT imaging score (= 133, 3), and rapidly progressive ILD (RPILD) (non-RPILD, 0; RPILD, 2). We divided patients into three risk groups according to the FLAIR score: low, 0 to 4; medium, 5 to 9; and high, 10 to 13. In both discovery and validation cohorts, high-risk patients had significantly higher mortality rates than low- and medium-risk patients (P < .001).INTERPRETATION: The FLAIR risk score model could help to predict survival in patients with ADM-ILD and to guide further clinical research on risk-based treatment.