An Analysis of Connective Tissue Disease-associated Interstitial Lung Disease at a US Tertiary Care Center: Better Survival in Patients with Systemic Sclerosis
An Analysis of Connective Tissue Disease-associated Interstitial Lung Disease at a US Tertiary Care Center: Better Survival in Patients with Systemic Sclerosis
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DOI：
10.3899/jrheum.100675
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发表时间：
2011-04-01
影响因子：
3.9
通讯作者：
Chung, Lorinda
中科院分区：
文献类型：
--
作者：
Su, Robert;Bennett, Mihoko;Chung, Lorinda
目标。比较结缔组织病相关间质性肺疾病(CTD-ILD)与特发性肺纤维化(IPF)和系统性硬化症相关性ILD(SSC-ILD)患者与其他CTD-ILD患者的生存率。我们使用了斯坦福ILD数据库，该数据库包含2002至2009年间在我们的三级护理中心评估的前瞻性收集的ILD患者的信息。最后用Kaplan-Meier估计法计算ILD确诊后的随访存活率。用单变量和多变量COX回归模型确定影响总体队列(IPF和CTD-ILD)和CTD-ILD组生存的预后因素。在427名ILD患者中，148名(35%)在基线访问时有IPF，76名(18%)有CTD-ILD。CTD累积发生率为4%。中位随访4年后，67例(36.4%)死亡，4例(2.2%)失访。IPF(n=122)和CTD-ILD(n=62)患者的生存率相似(5年生存率约为50%)。SSC-ILD患者(n=24)的生存率高于其他CTD-ILD患者(n=38)，1年、3年和5年生存率分别为100%、90%和77%，而其他CTD-ILD患者分别为78%、42%和38%(P=0.01)。在CTD-ILD患者中，即使在校正了ILD诊断时的年龄、性别和种族(HR=0.17，95%可信区间0.04-0.83)后，SSc的存在也将死亡风险降低了80%。SSC-ILD患者的生存率高于其他CTD-ILD患者，这可能与我们中心对SSC-ILD患者进行ILD的常规筛查和早期发现有关。(2011年2月1日首次发布；J Rheumatol 2011年；38：693-701；DOI：10.3899/jhurum.100675)
Objective. To compare survival of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) versus idiopathic pulmonary fibrosis (IPF) and patients with systemic sclerosis-associated ILD (SSc-ILD) versus other CTD-ILD followed at our center.Methods. We used the Stanford ILD database, which contains prospectively collected information on patients with ILL) evaluated at our tertiary care center from 2002 to 2009. Survival at last followup from time of ILD diagnosis was calculated using the Kaplan-Meier estimator. Prognostic factors-for survival in the overall cohort (IPF and CTD-ILD) and in the CTD-ILD group were identified with univariate and multivariate Cox regression models.Results. Of 427 patients with ILD, 148 (35%) had IPF and 76(18%) had CTD-ILD at the baseline visit. The cumulative incidence of CTD was 4%. After a median followup of 4 years, 67 patients (36.4%) had died and 4 (2.2%) were lost to followup. Patients with IPF (n = 122) and CTD-ILD (n = 62) experienced similar survival rates (5-year survival about 50%). Patients with SSc-ILD (n = 24) experienced better survival than those with other CTD-ILD (n = 38), with 1-year, 3-year, and 5-year survival rates of 100%; 90%, and 77%, respectively, versus 78%, 42%, and 38% (p = 0.01). The presence-of SSc in patients with CTD-ILD decreased the risk of death by > 80% even after correcting for age at ILD diagnosis, sex, and ethnicity (HR = 0.17, 95% CI 0.04-0.83).Conclusion. Survival in patients with SSc-ILD was better than in patients with other CTD-ILD, potentially related to routine screening for and early detection of ILD in patients with SSc at our center. (First Release Feb 1 2011; J Rheumatol 2011;38:693-701; doi:10.3899/jrheum.100675)