Hepatology Communications · 4 authors, 2 centres
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A retrospective single-center study of children and adolescents with cystic fibrosis found that lumacaftor/ivacaftor treatment significantly decreased serum markers of liver fibrosis in those with pre-existing CF-associated liver involvement, while elexacaftor/tezacaftor/ivacaftor did not show similar improvements.
This retrospective, single-center study analyzed children and adolescents with cystic fibrosis treated with lumacaftor/ivacaftor and/or elexacaftor/tezacaftor/ivacaftor, examining before- and during-treatment changes in liver function tests and fibrosis indices using previously established thresholds corresponding with increased liver elastography. In pairwise comparisons, patients with CF-associated liver involvement on lumacaftor/ivacaftor showed significant decreases in GGT, APRI, and GPR. These improvements were not observed in patients treated with elexacaftor/tezacaftor/ivacaftor or in patients without underlying CF-associated liver disease. The authors note that the lack of improvement with elexacaftor/tezacaftor/ivacaftor may be attributable to normalization of fibrosis markers during prior lumacaftor/ivacaftor exposure, as nearly all lumacaftor/ivacaftor patients achieved normalization of GGT and GPR. Neither cohort had baseline ultrasound or MRI elastography. The authors conclude lumacaftor/ivacaftor may represent the first drug to reverse CF-associated liver fibrosis, but note that larger multicenter studies with imaging or histology are needed for validation.
