Peptide and provisional matrix signals in idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a deadly disease presenting patients with few therapeutic options other than transplantation. Fibrotic lesions are found scattered throughout the lung parenchyma at different stages of progression. Some alveolar units are inflamed, others manifest epithelial denudation with fibroblastic foci, and still others are scarred shut with a mature collagenous matrix. For reasons that remain to be elucidated, fibrosis begins at the lung bases and periphery working its way upward and inward. Attempts to treat inflammation with the goal of interdicting fibrosis have been disappointing. The scope of research has therefore expanded, addressing signals that regulate lung fibroblast fate and alveolar reepithelialization. Such efforts may help us uncover new therapeutic targets.