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Prognosis of Patients With Advanced Idiopathic Pulmonary Fibrosis Requiring Mechanical Ventilation for Acute Respiratory Failure

Prognosis of Patients With Advanced Idiopathic Pulmonary Fibrosis Requiring Mechanical Ventilation for Acute Respiratory FailureIdiopathic pulmonary fibrosis (IPF) is a progressive fibrosing inflammatory lung disease of unknown etiology. The prognosis of the disease is particularly severe, with a mean length of survival after diagnosis ranging from 3 to 5 years. This poor prognosis is explained by the poor responsiveness to currently employed treatments, including steroids and cytotoxic agents. Studies2, evaluating new antifibrotic agents have yielded encouraging results, but the data are still preliminary. Additionally, patients with IPF may also now be considered for lung transplantation (LTx). Guidelines have been published, but the timing of referral for transplantation in such patients is not easy to define. Despite progress in the classification of IPF with the definition of several histologic patterns that are more responsive to therapy,- the prognosis in an individual patient remains variable. In some patients, the disease remains stable, whereas in others, life-threatening episodes of acute respiratory failure (ARF) are observed. In the latter patients, mechanical ventilation (MV) may be considered a therapeutic option, but the benefit offered using this strategy is not well documented. In our center, MV has been initiated on several occasions in patients with IPF developing ARF,
The aim of our study was to evaluate the beneficial effect of MV in this setting. To this purpose, we reviewed retrospectively all cases of patients with IPF who received MV for ARF in our center, with special emphasis on overall prognosis. The precipitating cause of ARF was also analyzed.
Materials and Methods

Hospital records of 27 consecutive patients with pulmonary fibrosis requiring MV for ARF admitted to our center between September 1990 and October 1999 were retrospectively examined. Our center is a 10-bed respiratory ICU that is a part of a respiratory department actively involved in LTx. The clinical diagnosis of IPF was based on the association of the three following criteria: (1) history of dyspnea and examination findings compatible with the diagnosis of IPF (bilateral crackles and/or clubbing); (2) chest radiograph and/or high-resolution CT scan showing typical pattern of IPF, such as ground-glass areas, irregular linear opacities, and honeycombing; and (3) no known cause of pulmonary fibrosis, such as hypersensitivity pneumonitis, connective tissue disease, drug or radiation-induced pneumonitis, or less frequent causes. Thus, four patients with pulmonary fibrosis secondary to radiation (n = 2), environmental exposure (n = 1), and sarcoidosis (n = 1) were excluded from analysis and the remaining 23 patients form the basis of the study.