ABSTRACT
Insights into the pathogenesis of idiopathic pulmonary fibrosis (IPF) and the strategies for treatment of this disorder have evolved over the past 30 years. In the pregenetic era, prior to the early 1970s, the pathogenesis and treatment of IPF was based on classic clinical and pathological assessments. In this period, the disease was defined on a clinical level, with the natural history, clinical presentation, blood chemistry, radiographic features, and morphology of the disorder understood (1-5). In what can be called the early genetic era, from the late 1970s to the present, as the newly developed technologies of mammalian molecular biology, cell biology, and culture were applied to lung disease, IPF was understood to be an inflammatory disorder with superimposed dysfunction of lung repair mechanisms, and new approaches to therapy were developed (6-10). In the current genetic era, with the sequencing of the human and murine genomes, the development of array-based strategies to evaluate gene expression, and the evolution of high-throughput sequencing and screening of drug libraries, the stage is set to make new insights into the pathogenesis of IPF and evolve new therapeutic approaches to the treatment of this disorder (11).
