Safety and extrapulmonary effects of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis following lung transplantation: A systematic review and meta-analysis.

BACKGROUND & AIMS: Elexacaftor-tezacaftor-ivacaftor is a cystic fibrosis transmembrane conductance regulator modulator used in people with cystic fibrosis. Currently, the paucity of efficacy data for extrapulmonary manifestations and concerns about adverse effects and drug interactions limit post-lung transplantation usage. This review aims to report the safety and efficacy of elexacaftor-tezacaftor-ivacaftor in people with CF after lung transplant.

METHODS

We conducted a systematic review through Pubmed, EMBASE and Scopus databases, selecting articles in English reporting clinical data on people with cystic fibrosis who received elexacaftor-tezacaftor-ivacaftor after lung transplant. Articles were scored using the Critical Appraisal Checklist for Case series and Cohort studies. A single-arm meta-analysis was performed from relevant studies to analyse change in ppFEV1, HbA1c, and BMI measurements when available.

RESULTS

Eight studies reported 195 cases. They reported benefits in terms of the complications associated with cystic fibrosis, such as poor nutritional status, diabetes, and chronic rhinosinusitis. Most lung transplant recipients tolerated elexacaftor-tezacaftor-ivacaftor well, although some suffered adverse events such as transaminase elevations and needed adjustments of immunosuppressive medications due to drug interactions.

CONCLUSION

Elexacaftor-tezacaftor-ivacaftor appears as a promising treatment in people with cystic fibrosis post-lung transplant, but prospective studies with long follow-up are needed to evaluate define indications and future applicability in people with cystic fibrosis while assessing the adverse effects and potential interaction with immunosuppressant medications.