Chest
Volume 120, Issue 1, July 2001, Pages 209-212
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Clinical Investigations in Critical Care
Outcome of Patients With Idiopathic Pulmonary Fibrosis Admitted to the ICU for Respiratory Failure

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Study objectives

To analyze the outcome of acuterespiratory failure (ARF) in patients with idiopathic pulmonaryfibrosis (IPF), and to evaluate the benefits of invasive andnoninvasive mechanical ventilation (MV).

Design

Retrospective study.

Setting

Universityhospital.

Patients

Fifteen consecutive patients with IPF referred to the ICU for ARF between January 1989 and June1998.

Measurements and results

Fifteen patients(mean ± SD age, 64 ± 10 years) were included. Eight patients hadclinical, functional, and radiologic features of IPF, and the remainingseven patients also had biopsy specimen-proven IPF. The mean durationbetween diagnosis of IPF and admission to the ICU was 26.5 ± 28months. At the time of ICU admission, mean arterial blood gas levelswere as follows: Pao2/fraction of inspiredoxygen, 113 ± 95; pH, 7.32 ± 0.10; and Paco2, 55 ± 21 mm Hg. All patients received MV; 12 patients required tracheal intubation, either at the time of ICUadmission (n = 10) or after failure of noninvasive ventilation (NIV;n = 2); and 3 patients only received NIV. Three of the five patientsreceiving NIV died of respiratory failure. Eleven patients died in the ICU, either from hypoxemia (n = 8) or from septic shock (n = 3). Four patients were discharged alive from the ICU, and two of them diedshortly thereafter.

Conclusion

The outcome ofpatients with IPF referred to the ICU for ARF was very poor and notimproved by MV. Without a clearly identified reversible cause of ARF, these patients should not benefit from admission to the ICU.

Section snippets

Selection of Patients

We retrospectively studied all consecutive patients with IPF who were referred to the respiratory ICU of Croix-Rousse Hospital, Lyon, France, for ARF, from January 1989 to June 1998. The definition of ARF was as follows: (1) exacerbation of dyspnea within a few days, (2) deterioration of hypoxemia (Pao2/fraction of inspired oxygen< 250), and (3) MV requirement. The diagnosis of IPF was based on a combination of the following criteria: persistent bilateral dry crackles on auscultation;

Characteristics of the Patients

Fifteen patients (11 men) were included (Table 1). Eight patients had clinical, functional, and radiologic features of IPF. Seven of them were known to have IPF at the time of admission, whereas in one patient the diagnosis of IPF was made during the ICU stay. In the seven remaining patients, the diagnosis of IPF was established by lung biopsy specimen, in addition to clinical symptoms; the biopsies were performed before ICU admission in six patients, and during ICU stay in one patient. In all

Discussion

In our study, the diagnosis of IPF was based on major clinical and radiologic criteria in eight patients10 and was definitely established by open-lung biopsy specimens in the remaining seven patients. Although surgical lung biopsy is the most reliable procedure to provide a definite diagnosis of IPF, it has been demonstrated that HRCT characteristics of IPF in an appropriate clinical context lead to a correct diagnosis in 85 to 90% of cases.5,8,15 The diagnosis of the ARDS could also be

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