Update in idiopathic pulmonary fibrosis

Abstract

This review aims to highlight and place in context recent advances in and insights into the natural history, diagnosis, and management of idiopathic pulmonary fibrosis (IPF).

Although the diagnosis of IPF remains challenging, an evolution in systems of practice and advancing technologies are steadily improving diagnostic accuracy. The identification of concomitant pulmonary hypertension as well as acute exacerbations of the underlying disease have taken on increasing importance in the natural history of IPF. Similarly, the management of IPF remains challenging, and although a number of recent trials of novel investigational agents for the treatment of IPF yielded negative results, at least one of these trials showed significant benefit suggesting progress in the treatment of this disease.

Although IPF remains a diagnostic and therapeutic challenge to even the most experienced of clinicians, our knowledge of the natural history of the disease, diagnostic accuracy, and therapeutic approach continue to advance.