ERS | monograph Preface Tobias Welte, Editor in Chief Although it is the most common genetic disease in Europe, cystic fibrosis (CF) is a rare disease. Nevertheless, it provides a good example of how a powerful patient organisation and dedicated researchers and doctors can help to arouse the interest of the public and the pharmaceutical industry in a disease. This was the key factor in maintaining continuous financial support to make translation from basic research into clinical practice possible. The success in the treatment of CF patients has contributed to significant improvements in the care of other patient groups. Essential elements of CF therapy have been transferred into the management of non-CF bronchiectasis patients and have recently been approved in randomised controlled trials. The best examples are three major macrolide antibiotic trials that demonstrate im- pressive effects on exacerbations in non-CF bronchiectasis patients. The concept of inhaled antibiotic therapy to reduce microbiological burden, which was established in CF, has now been used in studies in long-term ventilated patients with Gram-negative pneumonia and could provide a new treatment option for these patients, who still suffer from high morbidity and mortality. Along with pulmonary hypertension, CF is one of the few diseases where basic scientific findings have been translated into thera- peutic concepts. The modification of the cystic fibrosis trans- membrane conductance regulator (CFTR), even if it is possible only for particular mutations, represents a breakthrough in the development of new drugs. Through this, we have hopefully learned how dynamic basic research could be transferred into advanced therapies for other diseases, for example usual in- terstitial pneumonia, for which we do not have a convincing treatment option yet. Overall, CF research could be an excellent role model to stimulate research in respiratory medicine. This issue of the ERS Monograph summarises the most important developments in CF diagnosis and treatment since the publica- tion of the previous CF issue in 2006. I want to congratulate the Guest Editors, Marcus Mall and Stuart Elborn, for the tremendous work they have done in setting up this excellent Copyright ERS 2014. Print ISBN: 978-1-84984-050-7. Online ISBN: 978-1-84984-051-4. Print ISSN: 2312-508X. Online ISSN: 2312-5098. ERS Monogr 2014 64: ix–x. DOI: 10.1183/1025448x.10004414 ix
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