Pulmonary Complications of Cystic Fibrosis

Bryan Garcia, MD; Patrick A. Flume, MD


Semin Respir Crit Care Med. 2019;40(6):804-809. 

In This Article


Recent therapeutic advancements, including CFTR modulators, have resulted in significant improvements in life expectancy for patients with CF. The effect of these therapeutic advancements on the incidence and prevalence of complications in CF has not been studied and use of registry data will help better understand how modulators affect this aspect of CF lung disease. Nevertheless, despite these crucial therapeutic advancements, many patients with CF currently suffer from advanced lung disease or have significant pre-existing bronchiectasis and are expected to continue to decline clinically. For these reasons, pulmonologists and critical care providers that specialize in CF should be aware of the management of these frequently encountered complications and given the lack of randomized control trials into the management of these complications, as well as referral for lung transplantation, should be directed based on consensus guidelines.