Pulmonary Complications of Cystic Fibrosis

Bryan Garcia, MD; Patrick A. Flume, MD

Disclosures

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

In This Article

Abstract and Introduction

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.

Introduction

Cystic fibrosis (CF) is characterized by progressive loss of lung function due to the interplay of multiple factors including chronic airway infection, mucus stasis, and unabated pulmonary and systemic inflammation.[1] Therapeutic advancements including increased focus on optimizing nutrition through multivitamins and pancreatic enzymes, airway clearance therapies, antibiotics, and highly effective CF transmembrane conductance regulator (CFTR) modulators have resulted in improved health outcomes and improved survival for CF patients.[2–6] Despite this, many CF patients will continue to experience progressive obstructive lung disease and complications of pulmonary disease, including pulmonary exacerbations, acquisition of atypical respiratory pathogens, pneumothorax, hemoptysis, pulmonary hypertension, and the development of chronic hypoxic and hypercapnic respiratory failure.[7–9] The focus of this article is the care of CF patients with these pulmonary complications, with the exception of pulmonary exacerbations and atypical respiratory infections, which are further detailed elsewhere in this review series.

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