Impact of sleep-related hypoventilation in patients with pleuroparenchymal fibroelastosis

Yuki Yabuuchi; Takefumi Saito; Hitomi Hirano; Mizu Nonaka; Naoki Arai; Kentaro Hyodo; Jun Kanazawa; Yukiko Miura; Shingo Usui; Katsumi Tamura; Tomotaka Kasamatsu; Shuji Oh-Ishi; Kenji Hayashihara; Masashi Matsuyama; Nobuyuki Hizawa

doi:10.1186/s12931-022-02224-1

Impact of sleep-related hypoventilation in patients with pleuroparenchymal fibroelastosis

Respir Res. 2022 Oct 31;23(1):295. doi: 10.1186/s12931-022-02224-1.

Authors

Affiliations

¹ Department of Respiratory Medicine, National Hospital Organization, Ibaraki Higashi National Hospital, 825 Terunuma. Tokai-Mura, Naka-Gun, Ibaraki, 319-1113, Japan. jabbeflag@hotmail.co.jp.
² Department of Pulmonary Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan. jabbeflag@hotmail.co.jp.
³ Department of Respiratory Medicine, National Hospital Organization, Ibaraki Higashi National Hospital, 825 Terunuma. Tokai-Mura, Naka-Gun, Ibaraki, 319-1113, Japan.
⁴ Department of Clinical Research, National Hospital Organization, Ibaraki Higashi National Hospital, Ibaraki, Japan.
⁵ Department of Radiology, National Hospital Organization, Ibaraki Higashi National Hospital, Ibaraki, Japan.
⁶ Department of Radiology, National Defense Medical College, Saitama, Japan.
⁷ Department of Pulmonary Medicine, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan.

Abstract

Background: 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.

Methods: 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.

Results: 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).

Conclusions: 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.

Keywords: Chronic pulmonary aspergillosis; Non-invasive positive pressure ventilation; Partial pressure of carbon dioxide; Transcutaneous carbon dioxide.

MeSH terms

Connective Tissue Diseases*
Humans
Hypoventilation*
Lung
Sleep
Tomography, X-Ray Computed
Vital Capacity