Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
Department of Medicine, University of Saskatchewan, Saskatoon, SK, Canada.
Eur Respir Rev. 2023 Mar 8;32(167). doi: 10.1183/16000617.0210-2022. Print 2023 Mar 31.
Interstitial lung disease (ILD) is a frequent manifestation of connective tissue disease (CTD) with substantial variability in prevalence and outcomes reported across CTD subtypes. This systematic review summarises the prevalence, risk factors and ILD patterns on chest computed tomography of CTD-ILD.
A comprehensive search was performed in Medline and Embase to identify eligible studies. Meta-analyses were completed using a random effects model to determine the pooled prevalence of CTD-ILD and ILD patterns.
11 582 unique citations were identified with 237 articles included. Pooled prevalence of ILD was 11% in rheumatoid arthritis (95% CI 7-15%), 47% in systemic sclerosis (44-50%), 41% in idiopathic inflammatory myositis (33-50%), 17% in primary Sjögren's syndrome (12-21%), 56% in mixed connective tissue disease (39-72%) and 6% in systemic lupus erythematosus (3-10%). Usual interstitial pneumonia was the most prevalent ILD pattern in rheumatoid arthritis (pooled prevalence of 46%), while nonspecific interstitial pneumonia was the most common ILD pattern in all other CTD subtypes (pooled prevalence range 27-76%). Across all CTDs with available data, positive serology and higher inflammatory markers were risk factors for development of ILD.
We identified substantial variability in ILD across CTD subtypes suggesting that CTD-ILD is too heterogenous to be considered a single entity.
间质性肺病(ILD)是结缔组织病(CTD)的常见表现,在不同 CTD 亚型中,ILD 的患病率和结局存在很大差异。本系统综述总结了 CTD-ILD 的患病率、危险因素和胸部 CT 上的 ILD 模式。
在 Medline 和 Embase 中进行全面检索,以确定合格的研究。使用随机效应模型进行荟萃分析,以确定 CTD-ILD 的总患病率和 ILD 模式。
共确定了 11582 个独特的引文,其中包括 237 篇文章。ILD 的总患病率在类风湿关节炎中为 11%(95%CI7-15%),在系统性硬化症中为 47%(44-50%),在特发性炎症性肌病中为 41%(33-50%),在原发性干燥综合征中为 17%(12-21%),在混合性结缔组织病中为 56%(39-72%),在系统性红斑狼疮中为 6%(3-10%)。在类风湿关节炎中,最常见的 ILD 模式是寻常型间质性肺炎(总患病率为 46%),而在所有其他 CTD 亚型中,最常见的 ILD 模式是非特异性间质性肺炎(总患病率范围为 27-76%)。在所有有数据的 CTD 中,阳性血清学和更高的炎症标志物是发生 ILD 的危险因素。
我们发现 CTD 之间的 ILD 存在很大差异,这表明 CTD-ILD 过于异质,不能被视为单一实体。