Recent advances in the understanding and management of cystic fibrosis pulmonary exacerbations

Abstract

Pulmonary exacerbations are common events in cystic fibrosis and have a profound impact on quality of life, morbidity, and mortality. Pulmonary exacerbation outcomes remain poor and a significant proportion of patients fail to recover their baseline lung function despite receiving aggressive treatment with intravenous antibiotics. This focused review provides an update on some of the recent advances that have taken place in our understanding of the epidemiology, pathophysiology, diagnosis, and management of pulmonary exacerbations in cystic fibrosis as well as direction for future study.

Targeting Vitamin D Deficiency to Limit Exacerbations in Respiratory Diseases: Utopia or Strategy With Potential?

“…Definitions of acute exacerbations of chronic respiratory diseases vary according to the respiratory disease and exist based upon clinical findings. For cystic fibrosis (CF), the EuroCFCare Working group has recommended to use modified Fuchs criteria to define exacerbations of CF which include the need for additional antibiotic therapy and a recent change of at least two of the following criteria: change in sputum volume or color, increased cough, increased fatigue, malaise or anorexia, decrease in lung function by 10% or more, and increased dyspnea [2,3]. The Global Alliance of obstructive lung disease defines COPD exacerbations as an acute event characterized by worsening of the patient's respiratory symptoms that is beyond normal day-to-day variations and leads to a change in medication [4].…”

“…However, in all cases, the definition of exacerbation includes a subjective change in symptoms. Air pollution, some specific medication, smoking, chronic stress, and other triggers have been identified to cause exacerbations, but the most important trigger for exacerbations are respiratory infections caused by viruses or bacteria [3,6]. Pathogens enter the host by airborne transmissions (e.g., droplets or aerosols), replicate in the respiratory tract, and cause clinical worsening of symptoms (exacerbations).…”

Impact of the Local Inflammatory Environment on Mucosal Vitamin D Metabolism and Signaling in Chronic Inflammatory Lung Diseases

“…Chronic lung diseases are characterized by airway inflammation and impaired respiratory host defense, which is illustrated by the increased susceptibility for respiratory infections and exacerbations ( 25 , 80 , 81 ). Furthermore, exposure to inhaled toxicants such as cigarette smoke and air pollutants are associated with disease pathogenesis and exacerbations in COPD, CF and in asthma patients ( 82 – 84 ). It would therefore be of great interest to investigate these effects on local 1,25(OH) 2 D levels and on 1,25(OH) 2 D-mediated respiratory host defense in the airway mucosa.…”

Progressive Scoliosis in a Child with Cystic Fibrosis

“…In a patient with cystic fibrosis complaining of progressively worsening nasal drainage, cough, and fatigue, the differential includes a cystic fibrosis pulmonary exacerbation [6], which could be bacterial or viral in nature [7]. She had started treatment with an increased airway clearance regimen in order to reduce mucus plugging and was treated with oral antibiotics that were targeting her usual pulmonary microbiota.…”

“…She had started treatment with an increased airway clearance regimen in order to reduce mucus plugging and was treated with oral antibiotics that were targeting her usual pulmonary microbiota. Due to her continued symptoms on outpatient therapy, her pulmonary exacerbation could have been more significant and she failed outpatient therapy [7], or another process could be occurring.…”