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

https://doi.org/10.1016/j.jcf.2021.06.010Get rights and content
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Highlights

  • This is the first study to investigate intima-media thickness in cystic fibrosis (CF).

  • In pancreatic sufficient (PS) CF patients intima-media thickness was greater than in controls.

  • The link between intima-media thickness and LDL in PS CF may influence dietary guidelines.

  • Arterial stiffness was increased in CF as measured using digital volume pulse analysis.

  • Pancreatic insufficiency associated with arterial stiffness in CF, possibly because of inflammation and illness severity.

Abstract

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 × 10−5). IMT was increased in PS (but not PI) participants relative to control (552±69 µm vs 456±95 µm, 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 µm vs 453±82 µm, 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.

Keywords

Cystic fibrosis
Atherosclerosis
Arterial stiffness
Intima-media
Digital volume pulse
Lipids

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