title: Scleroderma-related lung disease: are adipokines involved pathogenically?
abstract: Scleroderma is a systemic autoimmune disease of unknown etiology whose characteristic features include endothelial cell dysfunction, fibroblast proliferation, and immune dysregulation. Although almost any organ can be pathologically involved in scleroderma, lung complications including interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the leading cause of death in patients with this condition. Currently, the molecular mechanisms leading to development of scleroderma-related lung disease are poorly understood; however, the systemic nature of this condition has led many to implicate circulating factors in the pathogenesis of some of its organ impairment. In this article we focus on a new class of circulating factors derived from adipose-tissue called adipokines, which are known to be altered in scleroderma. Recently, the adipokines adiponectin and leptin have been found to regulate biological activity in endothelial, fibroblast, and immune cell types in lung and in many other tissues. The pleiotropic nature of these circulating factors and their functional activity on many cell types implicated in the pathogenesis of ILD and PAH suggest these hormones may be mechanistically involved in the onset and/or progression of scleroderma-related lung diseases.
authors.affiliation: Center for Translational Medicine, Thomas Jefferson University, Philadelphia, PA, 19107, USA.
authors.forename: Shannon
authors.initials: S
authors.lastname: Haley
authors.forename: Dilip
authors.initials: D
authors.lastname: Shah
authors.forename: Freddy
authors.initials: F
authors.lastname: Romero
authors.forename: Ross
authors.initials: R
authors.lastname: Summer
mesh_terms: D054392:Adipokines / Q000502:physiology*; D052242:Adiponectin / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D020738:Leptin / Q000502:physiology; D017563:Lung Diseases, Interstitial / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology / Q000503:physiopathology
chemical_list: D054392:Adipokines; D052242:Adiponectin; D020738:Leptin