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Pneumothorax in Patients with Idiopathic Interstitial Pneumonias
Description
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A1685 - Pneumothorax in Patients with Idiopathic Interstitial Pneumonias
Author Block: M. Haraguchi1, M. Kato2, T. Nakamura2, K. Horikoshi2, H. Yoshikawa2, H. Ihara2, T. Asao2, S. Okamoto2, H. Takagi2, F. Takahashi2, M. Sekiya1, K. Seyama2, K. Takahashi2; 1Respiratory medicine, Saiseikai Kawaguchi General Hospital, Saitama, Japan, 2Department of Respiratory Medicine, Juntendo University Graduate School of Medicine, Tokyo, Japan.
Rationale
Pneumothorax is often observed as a complication of idiopathic interstitial pneumonias (IIPs). The incidence of pneumothorax in patients with idiopathic pulmonary fibrosis (IPF) has been reported to be 3.6-11%. The development of pneumothorax then influences their quality of life (QOL) because these patients usually experience severe dyspnea and chest pain. However, the clinical features in patients who concurrently present with IIPs and pneumothorax are unknown. We aimed to investigate the clinical features associated with the development of pneumothorax in patients with IIPs.
Methods
We recruited patients with IIPs who developed pneumothorax between April 2012 and October 2017 at both Juntendo University Hospital and Saiseikai Kawaguchi General Hospital. We retrospectively analyzed the clinical characteristics and prognosis of pneumothorax.
Results
We enrolled 23 patients with IIPs who developed pneumothorax. The median age in these patients was 69 years, and 19 patients were male. Seven patients exhibited usual interstitial pneumonia (UIP) patterns and were clinically diagnosed with IPF, 8 exhibited pleuroparenchymal fibroelastosis (PPFE) patterns, 2 were considered to exhibit non-specific interstitial pneumonia (NSIP) patterns, and the remaining patients were categorized as exhibiting unclassifiable IIP patterns based on chest high-resolution computed tomography (HRCT) findings. Four patients received pirfenidone, 6 were treated with corticosteroid, 3 received nintedanib, and the remaining patients were not treated with any agents for lung fibrosis. Chronic pneumothorax persisted in 9 patients after development of pneumothorax. The incidence of chronic pneumothorax in patients with PPFE was significantly higher than that in patients with other types of IIPs. Nineteen patients (86%) experienced more than two relapses of pneumothorax. The incidence of pneumothorax recurrence in patients with IIPs was higher than that in patients who developed spontaneous pneumothorax. Although 11 patients underwent thoracic drainage, 7 of these patients underwent pleurodesis and 3 of these 11 patients also underwent bullectomy. However, the success rate efficiency of medical treatment was about 18%. The median survival time in all patients who developed pneumothorax was 330 days from the development of pneumothorax. Eight patients died within 1 year after developing pneumothorax. Of these 8 patients, 4 patients died of worsening general conditions and the remaining patients died of acute exacerbation of IIPs.
Conclusion
The development of pneumothorax is caused by the worsening of respiratory conditions, QOL, and prognosis. Moreover, we should pay attention to the development of pneumothorax in patients with PPFE pattern on HRCT.
Author Block: M. Haraguchi1, M. Kato2, T. Nakamura2, K. Horikoshi2, H. Yoshikawa2, H. Ihara2, T. Asao2, S. Okamoto2, H. Takagi2, F. Takahashi2, M. Sekiya1, K. Seyama2, K. Takahashi2; 1Respiratory medicine, Saiseikai Kawaguchi General Hospital, Saitama, Japan, 2Department of Respiratory Medicine, Juntendo University Graduate School of Medicine, Tokyo, Japan.
Rationale
Pneumothorax is often observed as a complication of idiopathic interstitial pneumonias (IIPs). The incidence of pneumothorax in patients with idiopathic pulmonary fibrosis (IPF) has been reported to be 3.6-11%. The development of pneumothorax then influences their quality of life (QOL) because these patients usually experience severe dyspnea and chest pain. However, the clinical features in patients who concurrently present with IIPs and pneumothorax are unknown. We aimed to investigate the clinical features associated with the development of pneumothorax in patients with IIPs.
Methods
We recruited patients with IIPs who developed pneumothorax between April 2012 and October 2017 at both Juntendo University Hospital and Saiseikai Kawaguchi General Hospital. We retrospectively analyzed the clinical characteristics and prognosis of pneumothorax.
Results
We enrolled 23 patients with IIPs who developed pneumothorax. The median age in these patients was 69 years, and 19 patients were male. Seven patients exhibited usual interstitial pneumonia (UIP) patterns and were clinically diagnosed with IPF, 8 exhibited pleuroparenchymal fibroelastosis (PPFE) patterns, 2 were considered to exhibit non-specific interstitial pneumonia (NSIP) patterns, and the remaining patients were categorized as exhibiting unclassifiable IIP patterns based on chest high-resolution computed tomography (HRCT) findings. Four patients received pirfenidone, 6 were treated with corticosteroid, 3 received nintedanib, and the remaining patients were not treated with any agents for lung fibrosis. Chronic pneumothorax persisted in 9 patients after development of pneumothorax. The incidence of chronic pneumothorax in patients with PPFE was significantly higher than that in patients with other types of IIPs. Nineteen patients (86%) experienced more than two relapses of pneumothorax. The incidence of pneumothorax recurrence in patients with IIPs was higher than that in patients who developed spontaneous pneumothorax. Although 11 patients underwent thoracic drainage, 7 of these patients underwent pleurodesis and 3 of these 11 patients also underwent bullectomy. However, the success rate efficiency of medical treatment was about 18%. The median survival time in all patients who developed pneumothorax was 330 days from the development of pneumothorax. Eight patients died within 1 year after developing pneumothorax. Of these 8 patients, 4 patients died of worsening general conditions and the remaining patients died of acute exacerbation of IIPs.
Conclusion
The development of pneumothorax is caused by the worsening of respiratory conditions, QOL, and prognosis. Moreover, we should pay attention to the development of pneumothorax in patients with PPFE pattern on HRCT.
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