Mechanical Ventilation in Patients with End-Stage Idiopathic Pulmonary Fibrosis

Abstract

Background: Acute respiratory failure (ARF) occurring during idiopathic pulmonary fibrosis (IPF) is associated with a poor prognosis. In this subset of individuals, mechanical ventilation (MV) may be required. Objectives: We analysed the characteristics of a group of IPF patients undergoing MV for ARF in order to give some indications on the supposed prognosis. Methods: Hospital records of 34 consecutive patients with IPF, who underwent MV for ARF, were retrospectively examined. Demographic data, time from diagnosis, gas exchange, Acute Physiology and Chronic Health Evaluation (APACHE) II score, ARF causes and MV failure were recorded. Results: Fifteen subjects (group A) underwent invasive MV and 19 patients (group B) non-invasive ventilation (NIV). The 2 groups were different for disease severity (APACHE II score 24.2 ± 6 vs. 19.5 ± 5.9; p = 0.01). Both ventilatory strategies temporarily increased PaO₂/FiO₂ as compared with spontaneous breathing (group A: 148.5 ± 52 vs. 99 ± 39, p = 0.0004; group B: 134 ± 36 vs. 89 ± 26, p = 0.0004). NIV reduced the respiratory rate (26 ± 7 vs. 36 ± 9 with spontaneous breathing; p = 0.002). Duration of MV correlated with the time of evolution of IPF (r = 0.45; p = 0.018). The in-hospital mortality rate was 85% (100% for invasive MV, 74% for NIV). Four of the 5 survivors died within 6 months from hospital discharge (range 2–6 months). Conclusions: MV does not appear to have a significant impact on the survival of patients with end-stage IPF. NIV may be useful for compassionate use, providing relief from dyspnoea and avoiding aggressive approaches.

Recent lessons learned in the management of acute exacerbation of idiopathic pulmonary fibrosis

“…For patients treated with invasive (IMV) or noninvasive (NIV) mechanical ventilation, in-hospital mortality has been reported as 67% among 193 patients in studies published after 2008 (table 2) [44][45][46][47][48][49]. More recently, two national cohorts were reported.…”

Bridging to lung transplantation with extracorporeal circulatory support: when or when not?

“…This is especially true for patients who present with a rapid decline in respiratory status, requiring advanced airway support. These patients have a high rate of in-hospital mortality, and their candidacy for lung transplantation should be urgently assessed (1)(2)(3). Unfortunately, donor lungs are scarce, so bridging strategies may be necessary for survival.…”

Bleomycin-Induced Pulmonary Fibrosis, Severe Hypoxia and Intracardiac Air Embolism: Case Report

“…NIMV and IMV was shown to be similar in terms of mortality in case of need to mechanical ventilation support among patients with pulmonary fibrosis. 12,13 In our patient, due to development of tachypnea, hypoxia and hypercarbia despite the NIMV support within the first three days, IMV was initiated while peak and PEEP pressures were also required since hypoxia and hypercarbia were maintained under IMV. Use of high tidal volume after pulmonary fibrosis was shown to be associated with increase in lung injury and decrease in survival by triggering the inflammatory process.…”