Upregulation of elastin expression in constrictive bronchiolitis obliterans

Constrictive bronchiolitis obliterans is a fibrotic disease of small airways characterized by progressive obliteration of the airway lumen, with resulting obstructive pulmonary physiology. While previous work has demonstrated the collagenous nature of the constrictive fibrotic lesions, elastin expression in the disease has been poorly characterized. Elastin is a critical component of the pulmonary extracellular matrix, and is responsible for the reversible deformability characteristic of the alveoli, pulmonary blood vessels, and airways. Elastin is a long-lived protein with virtually no active protein production occurring after lung development is completed during early childhood. We report a novel case of cryptogenic bronchiolitis obliterans in which elastin gene expression is actively upregulated in affected airways, and accompanied by myofibroblast hyperplasia and disorganized elastic fiber deposition. In addition, deposition of new elastic fibers by myofibroblasts is noted in the alveoli surrounding the affected bronchioles.