Departments of Rheumatology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
Pneumology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.
Chest. 2020 Oct;158(4):1535-1545. doi: 10.1016/j.chest.2020.04.057. Epub 2020 May 16.
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.
How to identify patients with ADM-ILD who are at high risk and to predict patient outcome based on a risk stratification model?
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.
We developed a combined risk score, the FLAIR score, that included the following values and scores: ferritin (<636 ng/mL, 0; ≥636 ng/mL, 2), lactate dehydrogenase (<355 U/L, 0; ≥355 U/L, 2), antimelanoma differentiation-associated gene 5 antibody (negative, 0; +, 2; ++, 3; +++, 4), high-resolution CT imaging score (<133, 0; ≥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).
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.
无肌病性皮肌炎（ADM）相关性间质性肺病（ILD）的预后较差。需要建立一种死亡率风险评分模型来预测 ADM-ILD 患者的生存情况，并指导临床治疗。
如何识别 ADM-ILD 患者的高危人群，并基于风险分层模型预测患者预后？
我们对这项前瞻性发病研究中的 207 例 ADM-ILD 患者进行了评估。我们使用多变量 Cox 比例风险模型来确定独立的预后危险因素，并根据 2012 年 1 月至 2016 年 12 月患者的数据创建风险评分模型。我们使用预测准确性指数（Brier 评分）来反映模型的区分度和校准度。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。
我们开发了一个联合风险评分 FLAIR 评分，包括以下值和分数：铁蛋白（<636ng/mL，0；≥636ng/mL，2）、乳酸脱氢酶（<355U/L，0；≥355U/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（<133，0；≥133，3）和快速进展性ILD（RPILD）（非 RPILD，0；RPILD，2）。根据 FLAIR 评分，我们将患者分为三个风险组：低风险组（0-4 分）、中风险组（5-9 分）和高风险组（10-13 分）。在发现队列和验证队列中，高风险患者的死亡率均显著高于低风险和中风险患者（P<0.001）。
FLAIR 风险评分模型有助于预测 ADM-ILD 患者的生存情况，并指导基于风险的治疗的进一步临床研究。
