Interstitial lung diseases - where we started from and are now going

Background and Aim. Research into mechanisms causing interstitial lung diseases (ILD) began 35 years ago with the advent of cellular immunology and techniques to sample airways for biologic materials. After an analysis of lung research programs by the then National Heart and Lung Institute in 1972 identified as a priority the study of fibrotic and immunologic lung diseases, this began in the Pulmonary Branch (1974) of the Institute's intramural program. The Division of Lung Diseases initiated extramural research support also. ILD research developed quickly at many centers in the US and throughout the world. This review focuses on idiopathic pulmonary fibrosis (IPF) and highlights some of the initial research from the Pulmonary Branch. Recent research paradigm. In the 1990s research emphasis changed from a focus on inflammation to alveolar epithelial injury, fibrogenesis in fibroblastic foci, myofibroblast function, cytokine secretion and disordered matrix remodeling. More precise classification of ILD was advocated, especially for IPF. New strategies for therapy of IPF followed, including anti-fibrotic agents and interferon gamma treatment. However, therapy is still not sufficiently effective. Much is still left to do. Future Directions. The NHLBI research support continues for ILD, especially IPF. Current programs include: searching for new molecular therapeutic targets; establishing of a clinical network for IPF patients to assess combinations of therapy and new agents as appropriate; identifying genomic and genetic susceptibility factors; and creating a repository for lung tissue and biologic samples to aid investigators.