title: Pulmonary fibrosis: patterns and perpetrators.
abstract: Pulmonary fibrosis occurs in a variety of clinical settings, constitutes a major cause of morbidity and mortality, and represents an enormous unmet medical need. However, the disease is heterogeneous, and the failure to accurately discern between forms of fibrosing lung diseases leads to inaccurate treatments. Pulmonary fibrosis occurring in the context of connective tissue diseases is often characterized by a distinct pattern of tissue pathology and may be amenable to immunosuppressive therapies. In contrast, idiopathic pulmonary fibrosis (IPF) is a progressive and lethal form of fibrosing lung disease that is recalcitrant to therapies that target the immune system. Although animal models of fibrosis imperfectly recapitulate IPF, they have yielded numerous targets for therapeutic intervention. Understanding the heterogeneity of these diseases and elucidating the final common pathways of fibrogenesis are critical for the development of efficacious therapies for severe fibrosing lung diseases.
authors.affiliation: Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Medicine, Duke University School of Medicine, Durham, NC, USA. paul.noble@duke.edu
authors.forename: Paul W
authors.initials: PW
authors.lastname: Noble
authors.forename: Christina E
authors.initials: CE
authors.lastname: Barkauskas
authors.forename: Dianhua
authors.initials: D
authors.lastname: Jiang
mesh_terms: D000818:Animals; D020022:Genetic Predisposition to Disease; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000209:etiology / Q000473:pathology / Q000628:therapy; D008648:Mesoderm / Q000473:pathology; D008954:Models, Biological; D011650:Pulmonary Alveoli / Q000473:pathology; D011658:Pulmonary Fibrosis / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D020545:Respiratory Mucosa / Q000473:pathology