Iron Deficiency in Cystic Fibrosis: A Cross-Sectional Single-Centre Study in a Referral Adult Centre

Abstract

Iron deficiency (ID) diagnosis in cystic fibrosis (CF) is challenging because of frequent systemic inflammation. We aimed to determine the prevalence and risk factors of ID in adult patients with CF. We conducted a single-centre prospective study in a referral centre. ID was defined by transferrin saturation ≤ 16% or ferritin ≤ 20 (women) or 30 (men) µg/L, or ≤ 100 µg/L in the case of systemic inflammation. Apparent exacerbation was an exclusion criterion. We included 165 patients (78 women), mean age—31.1 ± 8.9 years. ID prevalence was 44.2%. ID was significantly associated with female gender (58.9% vs. 38%), lower age (29.4 ± 8.5 vs. 32.5 ± 9.1), lower body mass index (20.5 ± 2.2 vs. 21.3 ± 2.5), and Pseudomonas aeruginosa colonization (70.8% vs. 55.1%). Diabetes mellitus, antiacid drug use and low pulmonary function were more frequent in patients with ID with no statistical significance. The use of CFTR correctors was not associated with ID. In the multivariate analysis, ID was associated with female gender (OR 2.64, CI95% 1.31–5.31), age < 30 years (OR 2.30, CI95% 1.16–4.56), and P. aeruginosa (OR 2.09, CI95% 1.04–4.19).

Analysis of iron status after initiation of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis

“…7 Despite the uncertainty of pathogenesis, the rate of iron deficiency in PwCF has been predominantly reported as 60% with additional estimates ranging from 28% to 69%. 2,3,5,[8][9][10][11][12] Even when acute illness affecting laboratory results is accounted for, iron deficiency remains widespread across the CF population across all ages, genders, and disease severity. [2][3][4] However, no studies to date have focused on how cystic fibrosis transmembrane conductance regulator (CFTR) modulators impact the pathogenesis of iron deficiency.…”

“…Despite the uncertainty of pathogenesis, the rate of iron deficiency in PwCF has been predominantly reported as 60% with additional estimates ranging from 28% to 69% 2,3,5,8–12 . Even when acute illness affecting laboratory results is accounted for, iron deficiency remains widespread across the CF population across all ages, genders, and disease severity 2–4 .…”