Severe idiopathic pulmonary fibrosis: A clinical approach

Abstract

Idiopathic pulmonary fibrosis (IPF) is a devastating progressive disease associated with a high mortality rate. Novel antifibrotic therapies have been recently demonstrated to slow disease progression and improve survival. However, the management of IPF remains a difficult challenge, since lung complications can still occur, particularly in patients with advanced-stage disease. This paper highlights the most common complications and difficult tasks related to severe IPF such as acute exacerbation of the disease, development of lung cancer, rapid disease progression, and indication for lung transplantation.

Keywords: Acute exacerbation of IPF; Lung cancer; Lung transplantation; Nintedanib; Palliation; Pirfenidone.