Impact of sleep-related hypoventilation in patients with pleuroparenchymal fibroelastosis.
Impact of sleep-related hypoventilation in patients with pleuroparenchymal fibroelastosis.
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DOI：
10.1186/s12931-022-02224-1
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发表时间：
2022-10-31
影响因子：
5.8
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中科院分区：
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胸膜实质纤维弹性组织增生症（PPFE）是一种罕见的纤维化肺病，好发于肺上叶，其进展可引起肺通气不足，导致高碳酸血症。尽管睡眠相关通气不足（SRH）与慢性阻塞性肺疾病之间的关系已被充分证实，但其对PPFE患者的影响尚未评估。本研究的目的是阐明SRH对PPFE预后的影响。回顾性分析了52例在睡眠期间接受经皮二氧化碳监测的PPFE患者的病历。根据美国睡眠医学学会标准，将患者分为SRH组（n = 28）和非SRH组（n = 24）。评估了SRH对PPFE预后的影响，以及PPFE与SRH相关的临床因素和合并症。SRH组第一秒用力呼气量（FEV 1）、用力肺活量（FVC）、肺总容量（TLC）和一氧化碳弥散量（DLco）均明显低于非SRH组（P < .01）。慢性肺曲霉菌病（CPA）在SRH组中的发生率较高（P = .02）。SRH患者的中位生存时间为330天，而在3年的观察期内，大约80%的非SRH患者存活（P <0.01）。体重指数是PPFE伴SRH患者的一个重要预后因素（HR. 78; 95% CI;. 64-. 94; P <. 01）。白天家庭氧疗（HOT）和夜间睡眠时无创正压通气（NPPV）倾向于改善SRH组的预后，HR为.25（P = .07）。SRH可能是PPFE的不良预后因素。此外，SRH可能会改变PPFE患者对铜绿假单胞菌病的易感性。HOT联合NPPV可改善SRH患者的疾病结局。
Pleuroparenchymal fibroelastosis (PPFE) is a rare fibrosing lung disease with a predilection for the upper lobe and its progression causes hypoventilation, resulting in hypercapnia. Even though the association between sleep-related hypoventilation (SRH) and chronic obstructive pulmonary disease was well documented, its impact in patients with PPFE was not evaluated. The aim of this study is to clarify the impact of SRH on prognosis in PPFE. A retrospective review of the medical records of 52 patients with PPFE who underwent transcutaneous carbon dioxide monitoring during sleep was done. Patients were stratified into SRH (n = 28) and non-SRH (n = 24) groups based on American Academy of Sleep Medicine criteria. The impact of SRH on the prognosis of PPFE, as well as the clinical factors and comorbidities of PPFE associated with SRH, were evaluated. Forced expiratory volume in the first second (FEV1), forced vital capacity (FVC), total lung capacity (TLC), and carbon monoxide diffusing capacity (DLco) in the SRH group were significantly lower than the non-SRH group (P < .01). Chronic pulmonary aspergillosis (CPA) was found at a higher rate in the SRH group (P = .02). The median survival time for SRH patients was 330 days, whereas roughly 80% of non-SRH patients were alive during the 3-year observation period (P < .01). Body mass index was a significant prognostic factor in PPFE patients with SRH (HR .78; 95% CI; .64–.94; P < .01). Home oxygen therapy (HOT) during the day and noninvasive positive pressure ventilation (NPPV) at night while sleeping tended to improve prognosis in the SRH group, as indicated by HR of .25 (P = .07). SRH may be a poor prognostic factor for PPFE. Additionally, SRH may modify susceptibility to Aspergillosis in patients with PPFE. HOT plus NPPV may improve the disease outcomes in patients with SRH.