ELANE and pulmonary emphysema: In CPFE, neutrophil elastase and matrix metalloproteinases, enzymes physiologically involved in the destruction of bacteria by degrading the cell wall and in remodeling the cell–matrix by dissolving collagen, exceed the capacity of natural α1-antitrypsin inhibitors and lead to the destruction of alveolar septa rich in elastic fibers, resulting in emphysema [13].