A few scores predicting the short-term risk of mortality in Systemic sclerosis (SSc) have been reported to date. Our study aimed to create a predictive 15-year all-cause mortality score at the time of the diagnosis of SSc. The study was based on the Spanish Scleroderma Registry (RESCLE). The cohort was split up in derivation (DC) and validation cohort (VC). A multivariate analysis to detect variables related to all-cause mortality within the first 15 years from SSc diagnosis was performed, assigning points to the rounded beta values to create the score (RESCLESCORE). 1935 SSc patients were included. The variables in the final model were as follows: age at diagnosis (+2 points > 65 years-old), male gender (+1 point), lcSSc subset (-1 point), mode of onset other than Raynaud's (+1 point), cancer (+1 point) and visceral involvement, such as ILD (+1 point), PAH (+1 point), heart (+1 point) and renal involvement (+2 points). Autoantibodies did not achieve statistical significance in the multivariate analysis. The 3 categories of risk to predict 15-year all-cause mortality at the time of diagnosis were as follows: low risk (5% vs. 7%, p = .189), intermediate risk (26.5% vs. 25.5%, p = .911) and high risk (47.8% vs. 59%, p = .316). The AUC was 0.799 (DC) vs. 0.778 (VC) (p = .530). In conclusion, the RESCLESCORE demonstrated an excellent ability to categorize SSc patients at the time of diagnosis in separate 15-year all-cause mortality risk strata at the time of diagnosis.

中文翻译：

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西班牙硬皮病风险评分（RESCLESCORE）可根据RESCLE队列预测诊断时硬皮病患者15年全因死亡率：推导和内部验证。

迄今为止，已经有一些分数预测了系统性硬化症（SSc）的短期死亡风险。我们的研究旨在在诊断SSc时建立可预测的15年全因死亡率评分。该研究基于西班牙硬皮病登记处（RESCLE）。该队列分为派生（DC）和验证队列（VC）。进行了多变量分析以检测与SSc诊断后的前15年内的全因死亡率相关的变量，将点分配给四舍五入的beta值以创建分数（RESCLESCORE）。纳入1935名SSc患者。最终模型中的变量如下：诊断时的年龄（+2分> 65岁），男性（+1分），lcSSc子集（-1分），雷诺氏病以外的其他发作方式（+1分） ），癌症（+1分）和内脏受累，例如ILD（+1分），PAH（+1分），心脏（+1分）和肾脏受累（+2分）。自身抗体在多变量分析中未达到统计学意义。预测诊断时可预测15年全因死亡率的3种风险如下：低风险（5％vs. 7％，p = .189），中度风险（26.5％vs. 25.5％，p = .911）和高风险（47.8％vs. 59％，p = .316）。AUC为0.799（DC）对0.778（VC）（p = .530）。总之，RESCLESCORE在诊断时表现出了出色的能力，可以在诊断时将SSc患者分类为单独的15年全因死亡风险分层。预测诊断时可预测15年全因死亡率的3种风险如下：低风险（5％vs. 7％，p = .189），中度风险（26.5％vs. 25.5％，p = .911）和高风险（47.8％vs. 59％，p = .316）。AUC为0.799（DC）对0.778（VC）（p = .530）。总之，RESCLESCORE在诊断时表现出了出色的能力，可将SSc患者在诊断时分类为诊断时的单独的15年全因死亡率风险分层。预测诊断时可预测15年全因死亡率的3种风险如下：低风险（5％vs. 7％，p = .189），中度风险（26.5％vs. 25.5％，p = .911）和高风险（47.8％vs. 59％，p = .316）。AUC为0.799（DC）对0.778（VC）（p = .530）。总之，RESCLESCORE在诊断时表现出了出色的能力，可以在诊断时将SSc患者分类为单独的15年全因死亡风险分层。