Chest
Clinical Investigations: INTERSTITIAL LUNG DISEASEHistopathologic Features and Outcome of Patients With Acute Exacerbation of Idiopathic Pulmonary Fibrosis Undergoing Surgical Lung Biopsy
Section snippets
MATERIALS AND METHODS
We conducted a computer-assisted search of the Mayo Clinic database to identify patients with IPF who underwent surgical lung biopsy and were seen at our institution during a 7-year period from January 1, 1996, through December 31, 2002. Of 58 patients confirmed to have IPF by surgical lung biopsy, 7 patients (12%) underwent lung biopsy during an episode of acute exacerbation. None of these patients had an identifiable cause for their clinical deterioration other than their underlying lung
Demographic and Clinical Features
We identified seven patients with an acute exacerbation of IPF. The median age of these seven patients was 70 years (range, 59 to 74 years); two patients (29%) were women (Table 1). Five patients (71%) had a smoking history and included two current smokers. Four patients (57%) had the diagnosis of IPF established previously with a median interval of 15 months (range, 6 to 25 months) prior to presentation; three patients did not.
Four patients with previously diagnosed IPF had pulmonary function
DISCUSSION
A subset of patients with IPF experience acute exacerbation without an identifiable cause. To date, this variation on the natural history of IPF has been described only in patients with previously established diagnoses. In nearly half of our patients, this was instead the presenting manifestation of their previously unrecognized chronic interstitial lung disease. In most patients, IPF is a slowly but relentlessly progressive disease associated with a median survival of 2 to 3 years after
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Funding was provided by the Robert N. Brewer Family Foundation.
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