Lymphatics in idiopathic pulmonary fibrosis: new insights into an old disease.
The lymphatic vasculature plays a key role in tissue homeostasis and immune surveillance. There is mounting evidence of a role for the lymphatic circulation and for newly formed lymphatic vessels in the pathogenesis of lung disease. Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, debilitating lung disease. In IPF, the lung parenchyma undergoes extensive remodeling. This review focuses on the current knowledge and understanding of the pathogenesis of IPF, and recent evidence of the involvement of lymphangiogenesis in lung injury and repair and the molecular and cellular pathways leading to the development of lymphatic vasculature.
Translational Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892-1590, USA.
Souheil
S
El-Chemaly
Gustavo
G
Pacheco-Rodriguez
Yoshihiko
Y
Ikeda
Daniela
D
Malide
Joel
J
Moss
D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000473:pathology* / Q000628:therapy; D008208:Lymphatic System / Q000473:pathology*