Idiopathic pulmonary fibrosis (IPF) is a poorly understood progressive disease characterized
by the accumulation of scar tissue in the lung interstitium. A hallmark of the disease is areas
of injury to type II alveolar epithelial cells with attendant accumulation of fibroblasts in areas
called fibroblastic foci. In an effort to better characterize the lung fibroblast phenotype in IPF
patients, we isolated fibroblasts from patients with IPF and looked for activation of signaling
proteins, which could help explain the exaggerated fibrogenic response in IPF. We found …