To define the clinicopathologic features and outcome of acute exacerbations that occur in patients with idiopathic pulmonary fibrosis (IPF), a disorder that is generally characterized by slowly progressive course.
Retrospective single-center study.
The median age of these seven patients was 70 years (range, 59 years to 74 years); two were women. Four patients had a smoking history and included two current smokers. All patients were experiencing an exacerbation of dyspnea for a median duration of 14 days (range, 7 days to 28 days) prior to presentation. In three of these patients, the acute deterioration was the presenting feature of IPF while in the remaining four patients the diagnosis of IPF had previously been established. Chest radiography demonstrated bilateral mixed alveolar-interstitial infiltrates in all of them. Computed tomography revealed ground-glass opacities and consolidation bilaterally in all patients with associated peripheral honeycombing in six of them. Echocardiography was performed in six patients and demonstrated pulmonary hypertension in all. Bronchoalveolar lavage fluid was obtained in five patients and revealed neutrophilia in all. Surgical lung biopsy showed diffuse alveolar damage (DAD) in five patients with associated collagen fibrosis and honeycomb changes typical of usual interstitial pneumonia (UIP). One biopsy showed a combination of UIP and organizing pneumonia while one biopsy showed only DAD. Despite treatment with lung-protective ventilation strategies and high-dose systemic corticosteroids, six patients (86%) died during their hospitalization.
Although IPF is typically associated with an insidious, slowly progressive clinical course, acute exacerbations occur and may be the presenting manifestation in some patients. In either situation, current management strategies including high-dose corticosteroid therapy appear to be relatively ineffective and the mortality rate is high.
Acute exacerbations of IPF occur and may be the presenting or complicating manifestation in patients with IPF. In either situation, current management strategies including high-dose corticosteroid therapy appear to be relatively ineffective and the mortality rate is high.
Joseph Parambil, None.