Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease
Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease
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DOI：
10.2147/clep.s191418
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发表时间：
2019-01-01
影响因子：
3.9
通讯作者：
Verpillat, Patrice
中科院分区：
文献类型：
--
作者：
Bergamasco, Aurore;Hartmann, Nadine;Verpillat, Patrice
背景：间质性肺病（ILD）是系统性硬化症（SSc）患者死亡的主要原因之一。为了进一步了解这一患者人群，我们提出了第一个系统性审查的流行病学的SSc和SSc相关的ILD（SSc-ILD）的方法：文献数据库和网络资源进行了搜索，包括患者的研究与SSc和SSc-ILD在欧洲和北美（美国和加拿大）。系统性综述仅限于2000年1月1日至2016年2月29日期间发表的英文、德文、法文、西班牙文、意大利文和葡萄牙文出版物。对于纳入综述的所有出版物，评估了方法学质量。对于每个维度和地区，数据的可用性在数量和一致性的报告findings进行了evaluated.Results：50出版物报告的流行病学数据（患病率，发病率，人口统计学特征，生存率和死亡率），包括39例SSc患者和16例SSc-ILD患者。欧洲和北美报告的SSc患病率分别为每10万人7.2-33.9和13.5-44.3。欧洲和北美的年发病率估计分别为每10万人0.6-2.3和1.4-5.6。欧洲35%的患者和北美52%的患者存在相关ILD。在欧洲，一项研究估计SSc-ILD的患病率和年发病率分别为1.7-4.2和0.1-0.4/100，000个体。在欧洲和北美，SSc-ILD的诊断年龄比SSc略大，两种疾病的表现影响的女性是男性的2-3倍。据报道，在欧洲和54-82%在北美，与心肺表现（包括ILD）与预后不良SSc患者的10年生存率为65-73%，结论：本系统评价证实，SSc和SSc-ILD是罕见的，地理变异的患病率和发病率。
Background: Interstitial lung disease (ILD) is one of the leading causes of mortality in patients with systemic sclerosis (SSc). To further understand this patient population, we present the first systematic review on the epidemiology of SSc and SSc-associated ILD (SSc-ILD).Methods: Bibliographic databases and web sources were searched for studies including patients with SSc and SSc-ILD in Europe and North America (United States and Canada). The systematic review was limited to publications in English, German, French, Spanish, Italian, and Portuguese, published between January 1, 2000 and February 29, 2016. For all publications included in the review, the methodologic quality was assessed. For each dimension and region, data availability in terms of quantity and consistency of reported findings was evaluated.Results: Fifty publications reporting epidemiologic data (prevalence, incidence, demographic profile, and survival and mortality) were included; 39 included patients with SSc and 16 included patients with SSc-ILD. The reported prevalence of SSc was 7.2-33.9 and 13.5-44.3 per 100,000 individuals in Europe and North America, respectively. Annual incidence estimates were 0.6-2.3 and 1.4-5.6 per 100,000 individuals in Europe and North America, respectively. Associated ILD was present in similar to 35% of the patients in Europe and similar to 52% of the patients in North America. In Europe, a study estimated the prevalence and annual incidence of SSc-ILD at 1.7-4.2 and 0.1-0.4 per 100,000 individuals, respectively. In both Europe and North America, SSc-ILD was diagnosed at a slightly older age than SSc, with both presentations of the disease affecting 2-3 times more women than men. Ten-year survival in patients with SSc was reported at 65-73% in Europe and 54-82% in North America, with cardiorespiratory manifestations (including ILD) associated with poor prognosis.Conclusion: This systematic review confirms that SSc and SSc-ILD are rare, with geographic variation in prevalence and incidence.