Abstract

It is well demonstrated that inflammatory processes in the interstitium are pivotal as the pathological features of idiopathic interstitial pneumonia (IIP). Standard therapy with corticosteroids may suppress such inflammatory changes, but not result in marked clinical benefits, especially among patients with idiopathic pulmonary fibrosis (IPF). Recent studies clarified that the degrees of cell infiltration did not correlate with the clinical outcomes, but so-called remodeling processes, such as the number of fibroblastic foci, can more accurately predict the life expectancy among patients with IPF; the most common disease entity with poor prognosis. In accordance with such findings, anti-inflammatory strategies including treatment with corticosteroids, immunosuppressive agents, and even recently studied agent interferon-gamma have not been successful to stop or improve this process. More focuses should be now cast onto the molecular processes of myofibroblastic differentiation, proliferation and apoptosis of lung fibroblasts. Agents regulating these processes may become new therapeutic choices for these progressive pulmonary disorders.

Keywords: idiopathic interstitial pneumonia, idiopathic pulmonary fibrosis, lung remodeling, fibroblastic foci, myofibroblast, signal transduction