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Abstract
Background
It has been recognized that despite previous stability some patients with idiopathic pulmonary fibrosis (IPF) experience acute clinical deteriorations called acute exacerbations of idiopathic pulmonary fibrosis (AEX-IPF). We hypothesized that pulmonary infection can be excluded based on clinical and laboratory data and that bronchoscopy with BAL is not mandatory in the diagnostic work-up of suspected AEX-IPF.
Methods
In this retrospective study we identified patients with acute respiratory failure who were evaluated for AEX-IPF at the Cleveland Clinic between January 2002 and December 2011. Univariate and multivariate analysis were performed with predefined risk factors and final diagnosis of AEX-IPF and pulmonary infection. All tests were performed at a significance level of 0.05.
Results
A total of 77 patients met the study inclusion criteria. Of these patients 47 (61 %) were diagnosed with AEX-IPF. Bronchoscopy was more likely to be performed in patients who were on cytotoxic medications (p < 0.05). In most cases the diagnosis of AEX-IPF versus pulmonary infection was based on combination of other microbiological, clinical, radiologic data and clinical judgment. A total of 10 patients out of 14 (71 %) with a final diagnosis of pulmonary infection were on steroids on admission versus 21 out of 63 patients (33 %) with other final diagnosis (p = 0.024, OR 7.817, 95 % CI 1.31-46.64).
Conclusions
Exclusion of infection in our IPF patient cohort was mostly based on factors other than diagnostic bronchoscopy with BAL. Based on our results we suggested an algorithm for management of IPF patients presenting with acute respiratory failure.
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