Assessment of Progression Markers for Cirrhosis in Cystic Fibrosis Patients

Cystic Fibrosis is an autosomal dominant disease that affects the CFTR gene, which is responsible for producing a protein that regulates the transport of chloride ions across the cell membrane. This alteration results in obstruction in the body's ducts and channels, primarily affecting the pulmonary system. Additionally, other systems can be affected. Cystic Fibrosis-related liver disease is a common complication that can lead to steatosis, fibrosis, and multifocal biliary cirrhosis; however, there are no well-established diagnostic criteria yet. The present study was conducted at the Cystic Fibrosis Outpatient Clinic of the Hospital de Clínicas, Federal University of Paraná, examining 98 medical records of patients with cystic fibrosis for at least 10 years. Patients with hepatitis B, hepatitis C, alcohol consumption, or pregnancy were excluded. The objective was to determine the best predictor for the outcome of cirrhosis, and for this purpose, demographic parameters, laboratory tests, and imaging were collected. The statistical analysis was performed using SPSS® software, with quantitative variables described as mean ± standard deviation and categorical variables presented as the number of observations and percentage. ROC curves were adjusted to determine the best predictor, using the Youden index to establish the optimal cutoff point and the corresponding area under the curve to determine the model's efficacy. The study included 80 patients, and 10 of them (12.5%) were diagnosed with Cystic Fibrosis-related cirrhosis. The average age of the cirrhosis group was 21.6 years, with a predominance of males. The most accurate parameter for predisposition to cirrhosis was the APRI score, with a cutoff value of 0.27. Univariate analysis showed that gender, hepatic changes on ultrasound, splenomegaly, increased portal vein diameter, presence of vomiting, levels of alanine transaminase, aspartate transaminase, platelet count, and FIB-4 score were significantly associated with an APRI score value greater than 0.27. This study suggests the hypothesis that the APRI score is the most sensitive and specific tool for defining cirrhosis in patients with cystic fibrosis.