Impact of secondary spontaneous pneumothorax in chronic fibrosing interstitial lung disease Pneumothorax in chronic ILD

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Hideaki Yamakawa
Yuta Tsukahara
Shintaro Sato
Hiroki Ohta
Gen Kida
Tomohiko Nakamura
Tomotaka Nishizawa
Rie Kawabe
Tomohiro Oba
Keiichi Akasaka
Masako Amano
Kazuyoshi Kuwano
Hiroki Sasaki
Tamiko Takemura
Hidekazu Matsushima


pneumothorax, progressive fibrosing interstitial lung disease, prognosis


BACKGROUND: Secondary spontaneous pneumothorax (SSP) in interstitial lung disease (ILD) may influence prognosis of any ILD, and SSP onset predicts poor outcome in idiopathic pulmonary fibrosis (IPF). Recently, progressive fibrosing ILD (PF-ILD) has rapidly acquired importance.

OBJECTIVES: We hypothesized that the prognostic influence of SSP may be strongly affected in PF-ILD patients not limited to those with IPF.

METHODS: We retrospectively surveyed and collected data from the patients hospitalized for SSP from January 2016 to June 2020. PF-ILD was defined as the following occurring within 24 months before SSP develops: relative decline in %forced vital capacity (FVC) ≥10% or two of the following: relative decline in %FVC between 5% and 10%, worsening respiratory symptoms, or increased extent of fibrosis on high-resolution computed tomography.

RESULTS: We analyzed 32 patients hospitalized for SSP in ILD. This study comprised 18 patients with PF-ILD and 14 patients with non-PF-ILD. PF-ILD patients had lower body mass index (BMI) and %FVC. No significant differences in survival regarding follow-up period from the time of ILD diagnosis and hospitalization for SSP were observed between the PF-ILD and non-PF-ILD patients. Older age and lower BMI at were significant predictors of mortality by multivariate Cox regression analysis. ROC analysis showed BMI ≤17.8 kg/m2 to reliably predict poor prognosis.

CONCLUSIONS: Regardless of whether patients have PF-ILD, lower BMI in patients with ILD places them at higher risk of developing SSP and prognosis is poor if SSP develops. Therefore, clinical management of physique is important to improve the prognosis of ILD patients.

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1. Bobbio A, Dechartres A, Bouam S, et al. Epidemiology of spontaneous pneumothorax: gender-related differences. Thorax 2015; 70: 653-658.
2. Maskell N, British Thoracic Society Pleural Disease Guideline Group. British Thoracic Society Pleural Disease Guidelines--2010 update. Thorax 2010; 65: 667-669.
3. Nishimoto K, Fujisawa T, Yoshimura K, et al. The prognostic significance of pneumothorax in patients with idiopathic pulmonary fibrosis. Respirology 2018; 23: 519-525.
4. Nishimoto K, Fujisawa T, Yoshimura K, et al. Pneumothorax in connective tissue disease-associated interstitial lung disease. PLoS One 2020; 15: e0235624.
5. Travis WD, Costabel U, Hansell DM, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188: 733-748.
6. Yamakawa H, Kitamura H, Takemura T, et al. Prognostic factors and disease behaviour of pathologically proven fibrotic non-specific interstitial pneumonia. Respirology 2018; 23: 1032-1040.
7. Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 2019; 381: 1718-1727.
8. Tsukahara Y, Yamakawa H, Tsumiyama E, et al. Clinical features and prognosis of secondary pneumothorax in pulmonary emphysema, interstitial pneumonia, and combined pulmonary fibrosis and emphysema. Nihon Kokyuki Gakkai Zasshi 2020; 9: 160-165.
9. Wuyts WA, Wijsenbeek M, Bondue B, et al. Idiopathic pulmonary fibrosis: best practice in monitoring and managing a relentless fibrotic disease. Respiration 2020; 99: 73-82.
10. Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2018; 198: e44-68.
11. Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med 2018; 6: 138-153.
12. Ryerson CJ, Hartman T, Elicker BM, et al. Clinical features and outcomes in combined pulmonary fibrosis and emphysema in idiopathic pulmonary fibrosis. Chest 2013; 144: 234-240.
13. Huang TW, Cheng YL, Tzao C, et al. Factors related to primary bilateral spontaneous pneumothorax. Thorac Cardiovasc Surg 2007; 55: 310-312.
14. Iwasawa T, Ogura T, Takahashi H, et al. Pneumothorax and idiopathic pulmonary fibrosis. Jpn J Radiol 2010; 28: 672-679.
15. Lee JS, Collard HR, Raghu G, et al. Does chronic microaspiration cause idiopathic pulmonary fibrosis? Am J Med 2010; 123: 304-311.
16. Pugashetti J, Graham J, Boctor N, et al. Weight loss as a predictor of mortality in patients with interstitial lung disease. Eur Respir J 2018; 52: 1801289.
17. King TE Jr, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2083-2092.
18. Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071-2082.