Prognosis of acute exacerbation in idiopathic pulmonary fibrosis with pulmonary emphysema: a retrospective cohort study in Japan

BMJ Open. 2022 Sep 19;12(9):e062236. doi: 10.1136/bmjopen-2022-062236.

Abstract

Objectives: To analyse the clinical characteristics and prognosis of acute exacerbation (AE) in patients with idiopathic pulmonary fibrosis (IPF) and pulmonary emphysema.

Design: A multicentre retrospective cohort study SETTING: Two university hospitals in Japan PARTICIPANTS: Patients admitted to hospitals due to AE of IPF diagnosed based on a multidisciplinary discussion.

Interventions: None PRIMARY AND SECONDARY OUTCOME MEASURES: 90-day mortality rate METHODS: We retrospectively analysed consecutive patients with AE of IPF, with or without pulmonary emphysema, admitted to two university hospitals between 2007 and 2018.

Results: Among 62 patients (median age, 75 years; 48 men) admitted for AE of IPF, 29 patients (46%) presented with concomitant pulmonary emphysema. There was no significant difference in the arterial partial oxygen pressure/fraction of inhaled oxygen (P/F) ratio or other laboratory and radiographic data between patients with and without emphysema. The 90-day mortality rate was significantly lower in patients with emphysema than in those with IPF alone (23% vs 52%, p=0.03). The median survival time was significantly longer in patients with emphysema than in those with IPF alone (405 vs 242 days, p=0.02).

Conclusion: Patients with IPF and emphysema had better short-term survival after AE than those with non-emphysematous IPF.

Keywords: Adult intensive & critical care; Emphysema; Interstitial lung disease.

MeSH terms

  • Aged
  • Cohort Studies
  • Humans
  • Idiopathic Pulmonary Fibrosis* / complications
  • Japan
  • Male
  • Oxygen
  • Prognosis
  • Pulmonary Emphysema* / complications
  • Retrospective Studies

Substances

  • Oxygen