Study objectives: To define the clinicopathologic features and outcome of acute exacerbation in patients with idiopathic pulmonary fibrosis (IPF) undergoing surgical lung biopsy.
Design: Retrospective, single-center study.
Setting: Tertiary care, referral medical center.
Patients: Seven patients with acute exacerbation of IPF who underwent surgical lung biopsy.
Results: The median age of these seven patients was 70 years (range, 59 to 74 years); two were women. Five 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 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. CT 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. BAL 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.
Conclusions: 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 for these patients with acute exacerbation undergoing surgical lung biopsy.