An Acute and Life-Threatening Syndrome

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 ful¬ minating and life-threatening variant of this syndrome. Four patients presented with respiratory failure requir¬ ing respiratory assistance and positive pressure ventila¬ tion. Early recognition of the entity and prompt initia¬ tion of corticosteroid therapy in three patients was in¬ strumental 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 sus¬ picion for this entity. Early histologic diagnosis and initiation of corticosteroid therapy should be considered in patients with unexplained ARDS. (CHEST 1995; 108:271-77)