Chest
Volume 128, Issue 5, November 2005, Pages 3310-3315
Journal home page for Chest

Clinical Investigations: INTERSTITIAL LUNG DISEASE
Histopathologic Features and Outcome of Patients With Acute Exacerbation of Idiopathic Pulmonary Fibrosis Undergoing Surgical Lung Biopsy

https://doi.org/10.1378/chest.128.5.3310Get rights and content

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.

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

REFERENCES (18)

There are more references available in the full text version of this article.

Cited by (227)

  • Pathogenesis, Imaging, and Evolution of Acute Lung Injury

    2022, Radiologic Clinics of North America
  • Algorithmic Approach to the Diagnosis of Organizing Pneumonia: A Correlation of Clinical, Radiologic, and Pathologic Features

    2022, Chest
    Citation Excerpt :

    In ILDs, the OPP can be seen as (1) a minor lesion in the setting of ILDs, such as UIP, NSIP, or HP; (2) as a component of organizing acute lung injury, such as DAD; (3) as a manifestation of acute exacerbation of UIP or idiopathic pulmonary fibrosis or various other ILDs, including NSIP or HP, in which the underlying disease of one of these chronic ILDs is also present in a biopsy specimen and/or at CT scanning; and (4) in the setting of mixed patterns of lung injury, particularly NSIP and OP or eosinophilic pneumonia and OP. Distinguishing between OPP and eosinophilic pneumonia can be very difficult by means of CT scanning and/or biopsy, particularly if the patient has received corticosteroids before the biopsy, resulting in a significant reduction in the number of eosinophils in tissues.14-16,53–58 In cases in which the lesion appears to be predominantly that of the OPP, biopsy specimens should be evaluated for features suggestive of an underlying cause of SOP, such as prominent interstitial chronic inflammation (drug toxicity, collagen vascular disease [Fig 22]) or radiation pneumonitis (pneumocyte atypia).

View all citing articles on Scopus

Funding was provided by the Robert N. Brewer Family Foundation.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).

View full text