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Nontuberculous mycobacteria (NTM) are a group of mycobacteria representing opportunistic pathogens that are of increasing concern in people with cystic fibrosis (pwCF). The acquisition has been traditionally thought to be from environmental sources. However, recent work has suggested clustered clonal infections occur and transmission potential demonstrated among pwCF attending CF specialist centers. Guidelines for the screening, diagnosis, and identification of NTM and management of pwCF have been published. The emergence of CF-specific therapies, particularly cystic fibrosis transmembrane regulator (CFTR) modulator drugs, has led to significant improvements in the health and well-being of pwCF and may lead to challenges in sampling the lower respiratory tract, including screening for NTM. This review highlights the epidemiology, modes of acquisition, screening and diagnosis, therapeutic approaches in the context of improved clinical status for pwCF, and the clinical application of CFTR modulator therapies.
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