Chest
Volume 117, Issue 1, January 2000, Pages 282-285
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Selected Reports
Respiratory Bronchiolitis Associated With Severe Dyspnea, Exertional Hypoxemia, and Clubbing

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Respiratory bronchiolitis-associated interstitial lung disease(RBILD) is a distinct clinicopathologic disease described almostexclusively in cigarette smokers.1, 2 The disease usuallypresents with mild symptoms and is associated with a goodprognosis.2 Severe lung dysfunction has not been reportedwith RBILD, which is often confused clinically and radiographicallywith desquamative interstitial lung disease or idiopathic pulmonaryfibrosis (IPF). Two patients with RBILD who developed severe dyspnea, hypoxemia, and clubbing are described. Initially, IPF was diagnosed inboth patients. The severity of symptoms was such that the firstpatient's room air saturation was 85% and the second patient hadsevere impairment of lung function, with FEV1 of 39% andFVC of 40%. Advanced lung disease required supplemental home oxygentherapy in the first patient and referral for lung transplantevaluation in the second patient. After a detailed review of histologyrevealed a diagnosis of RBILD, both patients were encouraged to stopsmoking; smoking cessation led to considerable improvement in symptomsand lung function tests. We conclude that advanced lung dysfunctionoccurs in some patients with RBILD and should not dissuade thatdiagnosis.

Section snippets

Case 1

A 47-year-old previously well white man with a 25-year history of smoking had been treated at a local hospital for COPD and IPF for 4 years. The diagnosis of IPF was based on a chest radiograph, which showed reticular shadowing in both lung bases. He failed to improve with an empiric trial of steroids and continued to deteriorate to the point where he was unable to carry out his daily activities. Home oxygen therapy was necessary, and he was referred for further evaluation. Medications included

Case 2

A 43-year-old white man with a diagnosis of COPD and 100 pack-year history of tobacco use presented with increasing dyspnea of 2 years’ duration. He worked as a roofer until 2 years prior to presentation, when his symptoms worsened and he had to quit working. His medical history was significant only for an episode of hepatitis A. Medications included albuterol, ipratropium, and salmeterol inhalers. On examination, he had normal vital signs. Extremities revealed early clubbing of fingers.

Discussion

Cigarette smoking is a well-recognized cause of a variety of lung diseases, including COPD/emphysema, lung cancer, and respiratory bronchiolitis. Although interstitial fibrosis is recognized as a part of the histologic response to inhalation of cigarette smoke in both animals and humans,6, 7 the role of smoke inhalation as a cause of clinically significant interstitial lung disease is less well defined.

Respiratory bronchiolitis was first described in the early 1970s and is considered an

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This work was supported by The US Department of Veterans Affairs; and Grant No. HL 48164 and HL 07123, National Heart, Lung, and Blood Institute, National Institutes of Health.

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