A new study has assessed the prevalence and severity of liver disease among adults with cystic fibrosis (CF) and found both to be lower than expected.

The study, published in the Revista Española de Enfermedades Digestivas, based the assessment of liver disease on Debray and Koh criteria.

“Over the years, the diagnostic criteria for Cystic Fibrosis Liver Disease [CFLD] have been modified; nevertheless, the most accepted, to date, are those proposed by Debray et al,” the authors wrote. “Given that this pathology has not been thoroughly evaluated in adult patients and the limited number of publications on the subject, we decided to evaluate the prevalence, characteristics and potential risk factors of liver disease in adults with cystic fibrosis, according to two diagnostic criteria accepted in scientific literature.”

The research team recruited 95 adults 18 or more years of age with CF from a single center in Madrid, Spain, between January 2018 and January 2020. Clinical data on physical examinations and spirometry measurements were collected, blood samples were taken, and abdominal ultrasound and transient elastography were performed on all participants.

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CFLD was diagnosed per Debray and Koh criteria, and an elevation in at least two liver enzymes led to the diagnosis. The team found a 8.4% prevalence of liver disease, notably lower than the 25% reported in other studies.

In addition to the relatively low prevalence of CFLD in this patient group, the results found the course of the disease was relatively benign, with no terminal decompensated cirrhosis among the patients. The noninvasive liver fibrosis testing increased detection of CFLD, reinforcing its usefulness in clinical practice.

The authors suspect heterogeneity in the criteria for a diagnosis of CFLD contributed to the differences in prevalence in various studies. They recommend establishing a universally accepted criteria for improved characterization of the disease.


Marinero Martínez-Lázaro A, Girón Moreno RM, Casals Seoane F, et al. Cystic fibrosis with liver involvement in adults has a benign course. Results from a tertiary referral centre cohort. Rev Esp Enferm Dig. Published online November 10, 2022. doi:10.17235/reed.2022.9289/2022.