Chest
Volume 134, Issue 4, October 2008, Pages 844-850
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Selected Reports
Acute Exacerbations of Fibrotic Hypersensitivity Pneumonitis: A Case Series

https://doi.org/10.1378/chest.08-0428Get rights and content

Background

It is now recognized that a significant portion of patients with idiopathic pulmonary fibrosis (IPF) can have sudden and rapid deteriorations in disease course that cannot be explained by infection, heart failure, or thromboembolic disease. These events are often fatal and have been termed acute exacerbations (AEs) of underlying disease. While best described in patients with IPF, they have also been reported in patients with other forms of interstitial lung disease. We sought to determine if this same phenomenon occurs in patients with hypersensitivity pneumonitis (HP).

Methods

We retrospectively reviewed our clinical experience at National Jewish Medical and Research Center for patients with surgical lung biopsy-proven fibrotic HP who had an acute decline in respiratory status and met criteria similar to those proposed for the diagnosis of an AE of IPF.

Results

Over a 2-year period, we identified four patients with an AE of fibrotic HP. All patients had a clinical course similar to that most frequently described in AEs of IPF: respiratory failure requiring assisted ventilation, lack of clinical response to high-dose corticosteroid therapy, and a poor prognosis (all cases resulted in death or emergent lung transplantation). Lung biopsy at the time of the AE, explant, or autopsy revealed organizing diffuse alveolar damage superimposed on fibrotic lung disease.

Conclusions

Fibrotic HP, like other forms of fibrotic lung disease, can be associated with AEs of disease. Further investigation into similarities and pathways common in AEs of various fibrotic lung diseases may yield additional insight into this recently recognized syndrome.

Section snippets

Materials and Methods

We retrospectively reviewed the clinical files of patients undergoing consultation, evaluation, or treatment over a 2-year period (January 1, 2005, through January 1, 2007) at the Interstitial Lung Disease Program, National Jewish Medical and Research Center (Denver, CO). The Institutional Review Board approved this retrospective case series.

We sought out subjects with a clinico-radiographic-pathologic (via surgical lung biopsy) diagnosis of fibrotic HP,12, 13 a recent clinical course

Case 1

A 59-year-old woman with a 9-year history of biopsy-proven fibrotic HP secondary to mold exposure in her home environment was admitted to the hospital after 1 to 2 months of increasing dyspnea associated with a nonproductive cough. At the time of the initial diagnosis of fibrotic HP, she had moved out of her home, had the mold abated, and returned back to her home several months prior to the onset of the current symptoms. At the onset of symptoms, she was found to have new ground-glass

Discussion

In this report, we describe four patients with a history of biopsy-proven fibrotic HP who had an acute and accelerated decline in pulmonary status with new bilateral ground-glass opacities on HRCT. Despite an extensive evaluation for infection, cardiac dysfunction, and pulmonary embolism, no etiology for the precipitous deterioration was found. All patients met the recently proposed criteria for an AE-IPF, except these patients had a fibrotic lung disease other than IPF and the unexplained

Acknowledgment

The authors would like to express their gratitude to Carol Bair and Alma Kervitsky for their assistance with data collection.

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    This work was performed at National Jewish Medical and Research Center, Denver, CO.

    The authors have no conflicts of interest to disclose.

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

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