History of the use of CFTR modulators in the treatment of cystic fibrosis

Cystic fibrosis (CF) is the most common of the orphan lung diseases, well studied, not only highly sensitive diagnostic methods have been developed, cystic fibrosis centers with a multidisciplinary team and dynamic observation have been created, basic multicomponent therapy has been developed and targeted therapy has been connected for more than 10 years. The aim of the review was to study the history of CFTR modulators and evaluate their efficacy and safety. Based on the analysis of 79 scientific publications on targeted therapy for CF, an analysis of each drug was performed and a comparison of the efficacy of the last two most effective CFTR modulators, life expectancy and Vanzacaftor/tezacaftor/deitivacaftor. The primary and secondary indicators of the efficacy of CFTR modulators, adverse reactions, new problems associated with long-term use of drugs are discussed.