Cystic Fibrosis and Non-Cystic Fibrosis Bronchiectasis

The last few decades have seen remarkable achievements in clinical care in cystic fibrosis (CF), transforming it from a disease of childhood death into one of adult survival. In 2015, there are a greater proportion of adults than children with this condition. The reported prevalence of non-CF bronchiectasis has also increased over the last few years. In this issue of Seminars in Respiratory and Critical Care Medicine, international experts provide updates on recent advances within the fields of CF and non-CF bronchiectasis.

Prof. Noone and colleagues provide a comprehensive update on primary ciliary dyskinesia. Dr. Castellani and colleagues address the advances in understanding of the genotypes and phenotypes encountered in CF. Prof. Dodd and colleagues highlight the increasing importance of imaging modalities in providing a diagnosis, assessing complications, and evaluating disease progression and response to therapies in both CF and non-CF bronchiectasis.

Articles by Prof. Moss, Dr. Olivier, and colleagues, and Drs. Jones and Green highlight recent research that has significantly enhanced our understanding of the microbiology of CF and non-CF bronchiectasis. In addition to the traditionally recognized pathogens, such as Haemophilus influenza and Pseudomonas aeruginosa, other pathogens including fungi, nontuberculosis mycobacteria, and nonfermenting gram-negative bacteria are increasingly commonly encountered. Culture-independent studies have revealed that the lungs in CF and non-CF bronchiectasis are populated by a diverse microbiome.

CF is, of course, a multisystem disease. Drs. Brennan and Beynon address the pathophysiology and treatments for one of the most common extrapulmonary complications of CF, diabetes mellitus.

Articles by Dr. Main and colleagues, and Prof. Bell and colleagues, address the pivotal therapeutic strategies for the respiratory complications of CF and non-CF bronchiectasis: airway clearance, exercise, and inhaled antibiotics. Recently, there has been a major breakthrough in CF therapy, the success of small molecule modulators that address the basic defect in CF transmembrane regulator (CFTR) dysfunction. Prof. Plant and colleagues review the present and future therapies that aim to restore functional CFTR protein to patients.

Finally, despite the advances in evaluation and therapy for these conditions, many patients progress to end-stage respiratory failure and CF is one of the most common indications for lung transplantation. Prof. Lynch and colleagues discuss the indications, results, complications, and controversies of lung transplantation for CF.

As guest editor, I would like to thank all the authors for their enthusiasm and hard work, and the support of the Editor-in-Chief, Prof. Joseph Lynch, and the staff at Seminars in delivering an issue that provides an excellent comprehensive and expert insight into present and future advances in clinical care in CF and non-CF bronchiectasis.