Chest
Volume 108, Issue 1, July 1995, Pages 271-277
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Idiopathic Bronchiolitis Obliterans With Organizing Pneumonia: An Acute and Life-Threatening Syndrome

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Idiopathic bronchiolitis obliterans with organizing pneumonia (BOOP) is a clinicopathologic syndrome characterized by an indolent course and favorable prognosis. This report describes five patients with a fulminating and life-threatening variant of this syndrome. Four patients presented with respiratory failure requiring respiratory assistance and positive pressure ventilation. Early recognition of the entity and prompt initiation of corticosteroid therapy in three patients was instrumental in preventing mortality. Our findings suggest that idiopathic BOOP may be the underlying pathology in a number of patients presenting with ARDS. Since corticosteroid therapy may improve survival in these patients, clinicians should heighten their index of suspicion for this entity. Early histologic diagnosis and initiation of corticosteroid therapy should be considered in patients with unexplained ARDS.

Section snippets

Case 1

A 62-year-old woman presented to the emergency room with shortness of breath of 6 months’ duration, which had worsened over the last 2 to 3 days before admission. Fever and weight loss exceeding 20 lb were also reported. The patient denied cough, orthopnea, and paroxysmal nocturnal dyspnea. Her past medical history included diabetes mellitus, hypertension, and congestive heart failure for which she was taking insulin, fosinopril, furosemide, and potassium supplements. She had an 80 pack-year

The Clinical Picture

Signs and symptoms reported by our patients were nonspecific, constitutional, and mild (Table 2). Dyspnea was the most common complaint and was exertional in one instance. Cough productive of scanty whitish sputum was reported by four patients. All diagnostic studies done on bronchial secretions were uninformative. Pleuritic chest pain was observed in one patient. There was no associated pleural effusion or pneumothorax. Different patterns of radiologic pictures emerged and included diffuse,

Discussion

Bronchiolitis obliterans with organizing pneumonia secondary to viral infections, toxic exposures, the use of certain medications, cocaine abuse, and HIV infection has been clearly described in the literature.9, 10, 11 The idiopathic variety of this entity, however, was only first described in 1985 by Epler et al.1 Careful history and relevant investigations are required to differentiate the idiopathic variety of BOOP from the secondary causes of BOOP, which were just mentioned. Idiopathic

References (11)

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