Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors.
Aged
Asymptomatic Diseases
/ epidemiology
Female
Humans
Idiopathic Interstitial Pneumonias
/ complications
Idiopathic Pulmonary Fibrosis
/ complications
Japan
/ epidemiology
Lung
/ diagnostic imaging
Male
Pleura
/ diagnostic imaging
Pneumothorax
/ diagnosis
Residual Volume
Respiratory Function Tests
/ methods
Retrospective Studies
Severity of Illness Index
Survival Analysis
Thoracentesis
/ methods
Tomography, X-Ray Computed
/ methods
Total Lung Capacity
Idiopathic interstitial pneumonia
Pleuroparenchymal fibroelastosis
Pneumothorax
Journal
Respiration; international review of thoracic diseases
ISSN: 1423-0356
Titre abrégé: Respiration
Pays: Switzerland
ID NLM: 0137356
Informations de publication
Date de publication:
Historique:
received:
04
07
2020
accepted:
29
09
2020
pubmed:
6
1
2021
medline:
23
11
2021
entrez:
5
1
2021
Statut:
ppublish
Résumé
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.
Sections du résumé
BACKGROUND
BACKGROUND
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.
OBJECTIVE
OBJECTIVE
We aimed to investigate the complication of pneumothorax in patients with idiopathic PPFE.
METHODS
METHODS
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.
RESULTS
RESULTS
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.
CONCLUSION
CONCLUSIONS
Pneumothorax is often asymptomatic and recurrent in patients with idiopathic PPFE, leading to poor outcomes in some cases.
Identifiants
pubmed: 33401281
pii: 000511965
doi: 10.1159/000511965
doi:
Types de publication
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
19-26Informations de copyright
© 2021 S. Karger AG, Basel.