特发性肺纤维化合并肺气肿对结缔组织疾病和系统性硬化症患者的影响：系统评价和荟萃分析。
Effect of combined pulmonary fibrosis and emphysema on patients with connective tissue diseases and systemic sclerosis: a systematic review and meta-analysis.
机构信息
Department of Internal Medicine, Inje University Seoul Paik Hospital, Inje University College of Medicine, Seoul, South Korea.
Department of Physical Medicine and Rehabilitation, Ajou University School of Medicine, Suwon, South Korea.
出版信息
Arthritis Res Ther. 2021 Apr 6;23(1):100. doi: 10.1186/s13075-021-02494-y.
BACKGROUND
This study aimed to analyze the literature systematically to determine the clinical characteristics and prognosis of patients with connective tissue disease (CTD) with combined pulmonary fibrosis and emphysema (CPFE) compared to those of patients with CTD-interstitial lung disease (CTD-ILD) without emphysema.
METHODS
We searched MEDLINE, EMBASE, Cochrane Library, and KoreaMed for relevant articles published before July 2019. Studies meeting all the following criteria were included: (1) original research studies evaluating the effect of CPFE on CTD, (2) studies that compared patients with CTD-CPFE to those with CTD-ILD without emphysema, and (3) studies providing data on physical capacity, pulmonary function, or death in patients with CTD. Clinical characteristics of patients with CTD-CPFE were compared with those of patients with CTD-ILD without emphysema, and the influence of CPFE on physical capacity, pulmonary function, and death was analyzed.
RESULTS
Six studies between 2013 and 2019 were included. Two hundred ninety-nine (29.5%) and 715 (70.5%) patients had CTD-CPFE and CTD-ILD without emphysema, respectively. Regarding the type of CTD, 711 (68.3%) patients had systemic sclerosis, 263 (25.3%) rheumatoid arthritis, and 67 (6.4%) other CTDs. Patients with CTD-CPFE had a higher frequency of pulmonary hypertension and pulmonary fibrosis > 20% of the total lung volume, higher ratio of the forced vital capacity to the diffusion capacity of the lung for carbon monoxide (DLCO), lower arterial oxygen pressure at rest, and lower DLCO compared to those in patients with CTD-ILD without emphysema. In addition, more deaths occurred among those with CTD-CPFE (odds ratio, 2.95; 95% confidence interval, 1.75-4.96).
CONCLUSION
CTD-CPFE is associated with worse physical and pulmonary function and more deaths compared to those in CTD-ILD without emphysema. These findings indicate the need for increased awareness and close monitoring of patients with CTD-CPFE.
背景
本研究旨在通过系统分析文献，确定合并肺纤维化和肺气肿的结缔组织病（CTD）患者与无肺气肿的 CTD-间质性肺病（CTD-ILD）患者的临床特征和预后。
方法
我们检索了 MEDLINE、EMBASE、Cochrane 图书馆和 KoreaMed，以获取截至 2019 年 7 月前发表的相关文章。符合所有以下标准的研究被纳入：（1）评估 CPFE 对 CTD 影响的原始研究，（2）比较 CTD-CPFE 患者与无肺气肿的 CTD-ILD 患者的研究，以及（3）提供 CTD 患者体力、肺功能或死亡数据的研究。比较了 CTD-CPFE 患者的临床特征与无肺气肿的 CTD-ILD 患者，并分析了 CPFE 对体力、肺功能和死亡的影响。
结果
纳入了 2013 年至 2019 年的 6 项研究。299 例（29.5%）和 715 例（70.5%）患者分别患有 CTD-CPFE 和无肺气肿的 CTD-ILD。关于 CTD 类型，711 例（68.3%）患者为系统性硬化症，263 例（25.3%）为类风湿关节炎，67 例（6.4%）为其他 CTD。与无肺气肿的 CTD-ILD 患者相比，CTD-CPFE 患者更常发生肺动脉高压和肺纤维化占全肺总量的>20%、用力肺活量与一氧化碳弥散量的比值（DLCO）更高、静息时动脉血氧分压更低以及 DLCO 更低。此外，CTD-CPFE 患者的死亡人数更多（比值比，2.95；95%置信区间，1.75-4.96）。
结论
与无肺气肿的 CTD-ILD 患者相比，CTD-CPFE 患者的体力和肺功能更差，死亡人数更多。这些发现表明需要提高对 CTD-CPFE 患者的认识并加强对其的密切监测。
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