Preclinical atherosclerosis in cystic fibrosis: Two distinct presentations are related to pancreatic status

Background: Patients with cystic fibrosis (CF) are exposed to overlapping cardiovascular risk factors. We hypothesized that CF is characterized by increased arterial stiffness and greater intima-media thickness (IMT). Methods: This cross-sectional study assessed the digital volume pulse arterial stiffness index (SIDVp) using photopletysmography, measured intima-media complex thickness (IMT) at the common carotid artery, and obtained an extended set of clinical and atherosclerosis-related laboratory parameters. Results: Fifty-five patients with moderate-to-severe CF (mean age 26.3 +/- 8.6 years, BMI 20.3 +/- 3.1 kg/m2, FEV1 62 +/- 26%) and 51 healthy controls (25.1 +/- 4.4 years, BMI 21.7 +/- 3.0 kg/m2) entered the study. SIDVp was greater in pancreatic insufficient (PI), but not pancreatic sufficient (PS) CF patients compared with control (7.3 +/- 1.8 m/s vs 6.0 +/- 1.2 m/s; p=7.1 x 10(-5)). IMT was increased in PS (but not PI) participants relative to control (552 +/- 69 mu m vs 456 +/- 95 mu m, p=0.0011). SIDVp was also greater in PI than in PS patients (7.3 +/- 1.8 m/s vs 6.3 +/- 1.7 m/s, p=0.0232) and IMT was higher in PS compared with PI (552 +/- 69 mu m vs 453 +/- 82 mu m, p=0.0002). SIDVp independently associated with age, PI, the lack of liver cirrhosis, and with Pseudomonas aeruginosa colonization. PS was the only independent correlate of IMT in CF. Conclusions: PI patients are at risk of developing general arterial stiffness. PS may relate to carotid IMT thickening, which underscores the need for further study that could lead to reconsideration of dietary guidance in PS CF. (C) 2021 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Automated summary

Patients with cystic fibrosis (CF) have increased arterial stiffness and greater intima-media thickness (IMT), especially in those with pancreatic insufficient (PI) CF. The arterial stiffness index (SIDVp) is higher in PI CF patients compared to pancreatic sufficient (PS) CF patients and healthy controls. PS CF patients have higher IMT than PI CF patients and SIDVp is independently associated with age, PI, lack of liver cirrhosis, and Pseudomonas aeruginosa colonization.