Introduction Chronic pulmonary fibrosis PF) is a progressive inter- stitial lung disease characterized by fibroblast prolifer- ation and extracellular matrix remodeling [1]. This process may be secondary to systemic diseases [2] such as rheumatoid arthritis [3], systemic sclerosis [4], and sarcoidosis [5], but it may also be a primary pro- cess limited to the lung, and known as idiopathic pul- monary fibrosis IPF) [6]. The prevalence of IPF is low, and patients are usually 50 to 70 years old at pre- sentation [7]; secondary PF patients are more hetero- geneous in age [2]. Diagnosis is based on clinical histo- ry, high-resolution computed tomography HRCT), and histology, which shows usual interstitial pneumo- nia UIP) [1, 8]. IPF is a progressive disease, and no single therapy has been shown to alter the course of the disease [9]. Survival length from diagnosis varies between 3 and 5 years [10, 11], with a 5-year survival rate of 30±50 % [1]. Pulmonary transplantation has been proposed for IPF patients [12, 13], but its major limitations are the relatively advanced age of IPF patients in the terminal stages and organ availability. Secondary PF patients are a more heterogeneous population and may have a slightly better prognosis [14]. Thierry Fumeaux Claudia Rothmeier Philippe Jolliet Outcome of mechanical ventilation for acute respiratory failure in patients with pulmonary fibrosis Received: 9 May 2001 Final revision received: 10 September 2001 Accepted: 28 September 2001 Published online: 31 October 2001  Springer-Verlag 2001 T.Fumeaux  ) ) ´ P.Jolliet Division des Soins Intensifs de MØdecine, DØpartement de MØdecine, Hôpitaux Universitaires de Genve, Rue Micheli-du-Crest 24, 1211 Geneva 14, Switzerland E-mail: fumeauxt@usa.net Phone: +41-22-3 72 90 91 Fax: +41-22-3 72 91 05 C. Rothmeier Clinique de MØdecine 2, Hôpitaux Universitaires de Genve, Rue Micheli-du-Crest 24, 1211 Geneva 14, Switzerland Abstract Objective: During the course of idiopathic pulmonary fi- brosis patients may need invasive mechanical ventilation because of acute respiratory failure. We re- viewed the charts of all patients with idiopathic pulmonary fibrosis ad- mitted to our ICU for mechanical ventilation to describe their ICU course and prognosis. Design and setting: Retrospective, observational case series, from De- cember 1996 to March 2001, in an 18-bed medical ICU in a tertiary university hospital. Patients: Fourteen consecutive pa- tients with idiopathic n = 11) or secondary n = 3) pulmonary fibro- sis admitted to the medical ICU for mechanical ventilation. Measurements and results: Relevant factors of history and hospital course such as diagnostic and thera- peutic interventions were retrieved as well as laboratory and radiologi- cal results. All patients were admit- ted for severe acute hypoxemic re- spiratory failure PaO 2 /FIO 2 111  64 mmHg), with a high clinical suspicion of lower respiratory tract infection. Despite ventilatory sup- port and adjunctive therapies anti- biotics, steroids, or immunosuppres- sive drugs), all patients gradually worsened and eventually died in the ICU after a mean stay of 7.6  4.6 days. Conclusions: In this study mechani- cal ventilation for acute respiratory failure in pulmonary fibrosis pa- tients was associated with a 100 % mortality, despite aggressive thera- peutic and diagnostic procedures. Keywords Pulmonary fibrosis ´ Mechanical ventilation ´ Survival ´ Acute respiratory failure Intensive Care Med 2001) 27: 1868±1874 DOI 10.1007/s00134-001-1150-0 ORIGINAL