ABSTRACT
I. Introduction Cystic fibrosis (CF) lung disease is characterized by episodic increases in respiratory symptoms such as cough and sputum production, often accompanied by systemic symptoms such as anorexia and fatigue. These episodic changes in signs and symptoms from the patient’s baseline are termed pulmonary exacerbations (1,2). Pulmonary exacerbations have a significant impact on quality of life (3-6), mortality (7-10), and CF health care expenditures (11). Furthermore, they are amenable to therapies aimed both at prevention and treatment. The rate of pulmonary exacerbations has served as a key outcome measure in many clinical trials in CF over the past 15 years (12-18). This chapter reviews the pathophysiology, clinical characteristics, evaluation, treatment, and prevention of pulmonary exacerbations.
