Department of Internal Medicine, CHU Toulouse, Toulouse, Midi-Pyrénées, France.
INSERM UMR1027, 37 Allées Jules Guesdes, Toulouse, Midi-Pyrénées, France.
Arthritis Res Ther. 2021 Dec 7;23(1):295. doi: 10.1186/s13075-021-02672-y.
Systemic sclerosis (SSc) is associated with a variability of mortality rates in the literature.
To determine the mortality and its predictors in a long-term follow-up of a bi-centric cohort of SSc patients.
A retrospective observational study by systematically analyzing the medical records of patients diagnosed with SSc in Toulouse University Hospital and Ducuing Hospital. Standardized Mortality Ratio (SMR), mortality at 1, 3, 5, 10, and 15 years of disease and causes of death were described. Predictors of mortality using Cox regression were assessed.
Three hundred seventy-five patients were included: 63 with diffuse cutaneous SSc, 279 with limited cutaneous SSc, and 33 with sine scleroderma. The SMR ratio was 1.88 (95% CI 1.46-1.97). The overall survival rates were 97.6% at 1 year, 93.4% at 3 years, 87.1% at 5 years, 77.9% at 10 years, and 61.3% at 15 years. Sixty-nine deaths were recorded. 46.4% were SSc related deaths secondary to interstitial lung disease (ILD) (34.4%), pulmonary hypertension (31.2%), and digestive tract involvement (18.8%). 53.6% were non-related to SSc: cardiovascular disorders (37.8%) and various infections (35.1%) largely distanced those from cancer (13.5%). Four significant independent predictive factors were identified: carbon monoxide diffusing capacity (DLCO) < 70% (HR=3.01; p=0.0053), C-reactive protein (CRP) >5 mg/l (HR=2.13; p=0.0174), cardiac involvement (HR=2.86; p=0.0012), and the fact of being male (HR=3.25; p=0.0004).
Long-term data confirmed high mortality of SSc. Male sex, DLCO <70%, cardiac involvement, and CRP> 5mg/l were identified as independent predictors of mortality.
系统性硬化症（SSc）的文献报道死亡率存在差异。
通过对图卢兹大学医院和 Ducuing 医院的 SSc 患者进行双中心队列的长期随访，确定死亡率及其预测因素。
通过系统分析患者的病历，对在图卢兹大学医院和 Ducuing 医院诊断为 SSc 的患者进行回顾性观察性研究。描述标准化死亡率（SMR）、疾病 1、3、5、10 和 15 年的死亡率和死亡原因。使用 Cox 回归评估死亡率的预测因素。
共纳入 375 例患者：弥漫性皮肤型 SSc 63 例，局限性皮肤型 SSc 279 例，无皮肤型 SSc 33 例。SMR 比值为 1.88（95%CI 1.46-1.97）。1 年总生存率为 97.6%，3 年生存率为 93.4%，5 年生存率为 87.1%，10 年生存率为 77.9%，15 年生存率为 61.3%。记录到 69 例死亡。46.4%与 SSc 相关，死于间质性肺病（ILD）（34.4%）、肺动脉高压（31.2%）和消化道受累（18.8%）。53.6%与 SSc 无关：心血管疾病（37.8%）和各种感染（35.1%）在很大程度上降低了癌症（13.5%）的死亡率。确定了四个有意义的独立预测因素：一氧化碳弥散量（DLCO）<70%（HR=3.01；p=0.0053）、C 反应蛋白（CRP）>5mg/l（HR=2.13；p=0.0174）、心脏受累（HR=2.86；p=0.0012）和男性（HR=3.25；p=0.0004）。
长期数据证实 SSc 死亡率较高。男性、DLCO<70%、心脏受累和 CRP>5mg/l 是死亡率的独立预测因素。
