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Article ; Online: Eradication of Nontuberculous Mycobacteria in People with Cystic Fibrosis Treated with Elexacaftor/Tezacaftor/Ivacaftor: A Multicenter Cohort Study.

Wiesel, Vered / Aviram, Micha / Mei-Zahav, Meir / Dotan, Miri / Prais, Dario / Cohen-Cymberknoh, Malena / Gur, Michal / Bar-Yoseph, Ronen / Livnat, Galit / Goldbart, Aviv / Hazan, Guy / Hazan, Itai / Golan-Tripto, Inbal

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

2023  Volume 23, Issue 1, Page(s) 41–49

Abstract: Background: The prevalence of nontuberculous mycobacteria (NTM) infections is rising in people with cystic fibrosis (pwCF). NTM infection, especially infection with Mycobacterium abscessus complex (MABC), is commonly associated with severe lung ... ...

Abstract Background: The prevalence of nontuberculous mycobacteria (NTM) infections is rising in people with cystic fibrosis (pwCF). NTM infection, especially infection with Mycobacterium abscessus complex (MABC), is commonly associated with severe lung deterioration. The current treatment modalities, including multiple intravenous antibiotics, frequently fail to achieve airway eradication. Although treatment with elexacaftor/tezacaftor/ivacaftor (ETI) has been shown to modulate the lung microbiome, data regarding its role in eradicating NTM in pwCF is lacking. Our aim was to evaluate the impact of ETI on the rate of NTM eradication in pwCF.
Methods: This retrospective multicenter cohort study included pwCF from five CF centers in Israel. PwCF aged older than 6 who had at least one positive NTM airway culture in the past two years and were treated with ETI for at least one year were included. The annual NTM and bacterial isolations, pulmonary function tests, and body mass index were analyzed before and after ETI treatment.
Results: Fifteen pwCF were included (median age 20.9 years, 73.3% females, 80% pancreatic insufficient). In nine patients (66%) NTM isolations were eradicated following treatment with ETI. Seven of them had MABC. The median time between the first NTM isolation and treatment with ETI was 2.71 years (0.27-10.35 years). Eradication of NTM was associated with improved pulmonary function tests (p<0.05).
Conclusions: For the first time, we report successful eradication of NTM, including MABC, following treatment with ETI in pwCF. Additional studies are needed to assess whether treatment with ETI can result in the long-term eradication of NTM.
MeSH term(s) Female ; Humans ; Aged ; Young Adult ; Adult ; Male ; Nontuberculous Mycobacteria ; Cystic Fibrosis/complications ; Cystic Fibrosis/drug therapy ; Cystic Fibrosis/epidemiology ; Cohort Studies ; Mycobacterium abscessus ; Mycobacterium Infections, Nontuberculous/diagnosis ; Mycobacterium Infections, Nontuberculous/drug therapy ; Mycobacterium Infections, Nontuberculous/epidemiology ; Cystic Fibrosis Transmembrane Conductance Regulator ; Benzodioxoles ; Indoles ; Aminophenols ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones
Chemical Substances elexacaftor (RRN67GMB0V) ; ivacaftor (1Y740ILL1Z) ; tezacaftor ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6) ; Benzodioxoles ; Indoles ; Aminophenols ; Pyrazoles ; Pyridines ; Pyrrolidines ; Quinolones
Language English
Publishing date 2023-05-10
Publishing country Netherlands
Document type Multicenter Study ; Journal Article
ZDB-ID 2084724-5
ISSN 1873-5010 ; 1569-1993
ISSN (online) 1873-5010
ISSN 1569-1993
DOI 10.1016/j.jcf.2023.05.003
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