Semin Respir Crit Care Med 2019; 40(06): 804-809
DOI: 10.1055/s-0039-1697639
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Pulmonary Complications of Cystic Fibrosis

Bryan Garcia
1   Department of Medicine, Medical University of South Carolina, Charleston, South Carolina
,
Patrick A. Flume
1   Department of Medicine, Medical University of South Carolina, Charleston, South Carolina
2   Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina
› Author Affiliations
Further Information

Publication History

Publication Date:
28 October 2019 (online)

Abstract

Cystic fibrosis (CF) lung disease is characterized by the development of progressive bronchiectasis and impaired lung function with severe airflow obstruction. CF patients suffer from shortened life expectancy, primarily driven by respiratory failure. The mechanism by which CF lung disease develops is the result of an interplay of multiple intrinsic and extrinsic factors including genotype, abnormalities in mucus composition and movement, chronic inflammation, and chronic airway infection. Although all CF patients are at increased risk for pulmonary complications including hemoptysis, pneumothorax, pulmonary hypertension, and chronic hypoxic and hypercapnic respiratory failure, the risk of developing these complications increases with progression of lung disease. The focus of this article is to summarize the pathophysiology, epidemiology, and management of these key pulmonary complications.