Clinical Investigations

Mechanical Ventilation in Patients with End-Stage Idiopathic Pulmonary Fibrosis
Corrado Mollica^a, Gregorino Paone^{e, f}, Vittoria Conti^e, Daniela Ceccarelli^e, Giovanni Schmid^f, Paolo Mattia^b, Nicola Perrone^c, Angelo Petroianni^e, Alfredo Sebastiani^a, Luca Cecchini^a, Remo Orsetti^d, Claudio Terzano<sup>e

a</sup>Respiratory Intermediate Intensive Care Unit,
^bRadiological Unit,
^cUnit of Pulmonary Physiopathology, and
^dIntensive Care Unit, S. Camillo-Forlanini Hospital,
^eDepartment of Cardiovascular and Respiratory Sciences, University La Sapienza, and
^fThe Don Gnocchi Foundation S. Maria Della Pace, Rome, Italy

Address of Corresponding Author

Respiration 2010;79:209-215 (DOI: 10.1159/000225932)

  Key Words

• Acute respiratory failure
• Idiopathic pulmonary fibrosis
• Invasive mechanical ventilation
• Non-invasive mechanical ventilation

  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.

Copyright © 2009 S. Karger AG, Basel

  Author Contacts

Claudio Terzano
Department of Cardiovascular and Respiratory Sciences
Respiratory Diseases Unit, Sapienza University of Rome
Via Casal de’ Pazzi, 16, IT–00156 Rome (Italy)
Tel. +39 06 4080 0277, Fax +39 06 4080 0276, E-Mail cterzano@tin.it

  Article Information

C.M. and G.P. contributed equally to this work.

Received: August 11, 2008
Accepted after revision: March 25, 2009
Published online: June 17, 2009
Number of Print Pages : 7
Number of Figures : 2, Number of Tables : 2, Number of References : 32