Cacciapaglia Fabio, Airò Paolo, Fornaro Marco, Trerotoli Paolo, De Lorenzis Enrico, Corrado Addolorata, Lazzaroni Maria Grazia, Natalello Gerlando, Montini Fabio, Altomare Alberto, Urso Livio, Verardi Lucrezia, Bosello Silvia Laura, Cantatore Francesco Paolo, Iannone Florenzo
Department of Emergency and Organ Transplantations, Rheumatology Unit, University and AOUC Policlinico of Bari, Bari, Italy.
Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University, Brescia, Italy.
Rheumatology (Oxford). 2023 Apr 3;62(4):1552-1558. doi: 10.1093/rheumatology/keac512.
Survival and death prognostic factors of SSc patients varied during the past decades. We aimed to update the 5- and 10-year survival rates and identify prognostic factors in a multicentre cohort of Italian SSc patients diagnosed after 2009.
Patients who received a diagnosis of SSc after 1 January 2009 and were longitudinally followed up in four Italian rheumatologic centres were retrospectively assessed up to 31 December 2020. Overall survival of SSc patients was described using the Kaplan-Meier method. Predictors of mortality at 10-year follow-up were assessed by the Cox regression model. A comparison of our cohort with the Italian general population was performed by determining the standardized mortality ratio (SMR).
A total of 912 patients (91.6% females, 20% dcSSc) were included. Overall survival rates at 5 and 10 years were 94.4% and 89.4%, respectively. The SMR was 0.96 (95% CI 0.81, 1.13), like that expected in the Italian general population. Pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) associated with pulmonary hypertension (PH) significantly reduced survival (P < 0.0001). Main death predictors were male gender (HR = 2.76), diffuse cutaneous involvement (HR = 3.14), older age at diagnosis (HR = 1.08), PAH (HR = 3.21), ILD-associated PH (HR = 4.11), comorbidities (HR = 3.53) and glucocorticoid treatment (HR= 2.02).
In the past decade, SSc patients have reached similar mortality of that expected in the Italian general population. Male gender, diffuse cutaneous involvement, comorbidities and PAH with or without ILD represent the main poor prognostic factors.
在过去几十年间，系统性硬化症（SSc）患者的生存及死亡预后因素有所变化。我们旨在更新5年和10年生存率，并在2009年后诊断的意大利SSc患者多中心队列中确定预后因素。
对2009年1月1日后确诊为SSc且在四个意大利风湿病中心进行纵向随访的患者进行回顾性评估，直至2020年12月31日。采用Kaplan-Meier法描述SSc患者的总生存率。通过Cox回归模型评估10年随访时的死亡预测因素。通过确定标准化死亡率（SMR）将我们的队列与意大利普通人群进行比较。
共纳入912例患者（女性占91.6%，弥漫性皮肤型SSc占20%）。5年和10年的总生存率分别为94.4%和89.4%。SMR为0.96（95%可信区间0.81, 1.13）,与意大利普通人群预期相似。肺动脉高压（PAH）以及与肺动脉高压（PH）相关的间质性肺疾病（ILD）显著降低生存率（P < 0.0001）。主要的死亡预测因素为男性（风险比[HR]=2.76）、弥漫性皮肤受累（HR = 3.14）、诊断时年龄较大（HR = 1.08）、PAH（HR = 3.21）、与ILD相关的PH（HR = 4.11）、合并症（HR = 3.53）以及糖皮质激素治疗（HR = 2.02）。
在过去十年中，SSc患者的死亡率与意大利普通人群预期相似。男性、弥漫性皮肤受累、合并症以及伴有或不伴有ILD的PAH是主要的不良预后因素。
