Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan,
Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Japan,
Respiration. 2021;100(1):19-26. doi: 10.1159/000511965. Epub 2021 Jan 5.
Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare form of idiopathic interstitial pneumonia that is characterized by predominantly upper lobe pleural and subpleural lung parenchymal fibrosis. Pneumothorax is one of the major respiratory complications in PPFE patients; however, its clinical features are poorly understood.
We aimed to investigate the complication of pneumothorax in patients with idiopathic PPFE.
A retrospective multicenter study involving 89 patients who had been diagnosed with idiopathic PPFE was conducted. We investigated the cumulative incidence, clinical features, and risk factors of pneumothorax after the diagnosis of idiopathic PPFE.
Pneumothorax developed in 53 patients (59.6%) with 120 events during the observation period (41.8 ± 35.0 months). The cumulative incidence of pneumothorax was 24.8, 44.9, and 53.9% at 1, 2, and 3 years, respectively. Most events of pneumothorax were asymptomatic (n = 85; 70.8%) and small in size (n = 92; 76.7%); 30 patients (56.6%) had recurrent pneumothorax. Chest drainage was required in 23 pneumothorax events (19.2%), and a persistent air leak was observed in 13 (56.5%). Patients with pneumothorax were predominantly male and frequently had pathological diagnoses of PPFE and prior history of pneumothorax and corticosteroid use; they also had significantly poorer survival than those without pneumothorax (log-rank test; p = 0.001). Multivariate analysis revealed that a higher residual volume/total lung capacity ratio was significantly associated with the development of pneumothorax after the diagnosis.
Pneumothorax is often asymptomatic and recurrent in patients with idiopathic PPFE, leading to poor outcomes in some cases.
特发性胸膜肺弹力纤维增生症（PPFE）是一种罕见的特发性间质性肺炎，其特征为主要在上肺胸膜和肺实质下区纤维化。气胸是 PPFE 患者的主要呼吸并发症之一；然而，其临床特征尚未被充分了解。
我们旨在研究特发性 PPFE 患者气胸的并发症。
进行了一项回顾性多中心研究，纳入了 89 名被诊断为特发性 PPFE 的患者。我们调查了特发性 PPFE 诊断后气胸的累积发生率、临床特征和危险因素。
在观察期间（41.8±35.0 个月），53 名（59.6%）患者发生了 120 次气胸事件。气胸的累积发生率分别为 1 年、2 年和 3 年时的 24.8%、44.9%和 53.9%。大多数气胸事件为无症状（n=85；70.8%）和小量气胸（n=92；76.7%）；30 名（56.6%）患者发生复发性气胸。23 次气胸事件需要行胸腔引流（19.2%），13 次（56.5%）气胸存在持续漏气。气胸患者主要为男性，常伴有特发性 PPFE 病理诊断、气胸病史和皮质类固醇使用史；与无气胸患者相比，他们的生存率显著更差（对数秩检验；p=0.001）。多变量分析显示，诊断后残气量/肺总量比值较高与气胸的发生显著相关。
特发性 PPFE 患者的气胸常为无症状和复发性，在某些情况下导致不良结局。
