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Influence of interstitial lung disease on outcome in systemic sclerosis: A population-based historical cohort study

机译:间质性肺疾病对系统性硬化症结局的影响:一项基于人群的历史队列研究

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Background: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and a major cause of SSc-related deaths. This study aimed to determine the infl uence of ILD on SSc in a population-based historical cohort study. The hypothesis was that patients with SSc who develop ILD have increased morbidity and mortality when compared with patients with SSc without ILD. Methods: Using the record linkage system of the Rochester Epidemiology Project in Olmsted County, Minnesota, this study identifi ed the incidence of SSc between 1980 and 2010 and point prevalence on December 31, 2010 and determined the progression of organ involvement and its infl uence on outcome. Results: During the 30-year interval, we identifi ed 64 incident cases of SSc: 57 women and seven men, median age 49.1 years (interquartile range [IQR], 39.8-67.6 years). There were 43 prevalent cases. ILD occurred in 19 cases, usually after the diagnosis of SSc (median, 2 years; IQR, 0-10 years), with only three cases occurring 6 to 24 months beforehand. Pulmonary arterial hypertension (PAH) was diagnosed in 14 cases, heart failure in 27 cases, and chronic kidney disease (CKD) in 21 cases. Seventeen patients died during the study period, with a median survival time after diagnosis of 22.9 years. ILD, PAH, and CKD were associated with an increased risk of death. Conclusions: The incidence of ILD associated with SSc was relatively low in this population-based cohort. ILD appeared to be a contributing factor to mortality. Other factors, including age, PAH, and CKD, were also associated with poor outcome.
机译:背景:间质性肺疾病(ILD)是系统性硬化症(SSc)的常见并发症,是与SSc相关的死亡的主要原因。这项研究旨在确定基于人群的历史队列研究中ILD对SSc的影响。假设是与没有ILD的SSc患者相比,发生ILD的SSc患者的发病率和死亡率增加。方法:本研究使用明尼苏达州奥尔姆斯特德县罗切斯特流行病学项目的记录链接系统,确定了1980年至2010年间SSc的发病率和2010年12月31日的点流行率,并确定了器官受累的进展及其影响。结果。结果:在30年的时间间隔内,我们确定了64例SSc的发病病例:57名女性和7名男性,中位年龄49.1岁(四分位间距[IQR],39.8-67.6岁)。有43例流行病例。 ILD发生于19例,通常在确诊为SSc之后(中位2年; IQR,0-10年),只有3例发生在6至24个月之前。诊断为肺动脉高压(PAH)14例,心力衰竭27例,慢性肾脏疾病(CKD)21例。在研究期间,有17名患者死亡,诊断后中位生存时间为22.9年。 ILD,PAH和CKD与死亡风险增加相关。结论:在这个基于人群的队列研究中,与SSc相关的ILD发生率相对较低。 ILD似乎是导致死亡的一个因素。其他因素,包括年龄,PAH和CKD,也与不良预后相关。

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