ABSTRACT
The management of idiopathic pulmonary fibrosis (IPF) has evolved little over decades. The development of novel treatments was limited by an incomplete knowledge of the underlying pathophysiology and epidemiology. Recently there have been a number of new insights, both epidemiological and histopathological, that will likely impact on how we currently approach the management of IPF. Studies now suggest that IPF is more common, with both the incidence and prevalence of IPF being up to 10 times higher than originally supposed (1). Also, we now recognize that IPF is a distinct clinical entity associated with the histopathological pattern of usual interstitial pneumonia. Pathologically, IPF is characterized by inflammation, fibroblastic proliferation, and collagen deposition, with the current most prevalent focus being on fibrogenesis and the concept of aberrant wound healing. With regard to treatment options, we are currently at a crossroads as we move away from traditional anti-inflammatory therapies that have had, disappointingly, only minimal impact on improving disease outcome, toward a more targeted antifibrotic approach. As our understanding of the pathogenesis of lung fibrosis has evolved, we are now better positioned to develop future interventional strategies. However, despite an increased understanding of the cellular and molecular mechanisms underlying IPF and the emergence of a number of more targeted treatments, we still have no proven therapy for IPF.
