Researchers discovered that the modified aspartate aminotransferase-to-platelet ratio index score (mAPRI) is a highly specific and noninvasive tool for the diagnosis of cystic fibrosis-related liver disease (CFRLD), as published in Clinical Transplantation.

Despite the fact that liver disease is a significant cause of morbidity and mortality in patients with cystic fibrosis (CF), there is still no standard criterion for the diagnosis of CFRLD. In addition, scientists still do not know why some patients with CF have significant liver disease while others do not.

Patients with CFRLD may have varying degrees of liver damage, ranging from an abnormal liver profile with relatively few symptoms to significant liver damage requiring liver transplantation.

Patients with CF often require lung transplantation; studies have demonstrated that it improves the quality of life and survival. However, the impact of CFRLD on lung transplantation is still unclear.

Read more about CF comorbidities

The authors of this study hypothesized that the mAPRI can be used to diagnose CFRLD. The mAPRI is already in use to quantify liver fibrosis in a noninvasive way. They retrospectively reviewed the experience of 238 patients with CF who underwent double lung transplantation. Patients were divided into 2 groups: those with and without CFRLD. The mAPRI was calculated for each patient and used to predict the severity of the liver disease.

“We have shown that a mAPRI score of greater than 0.2 has a sensitivity of 43.0% and specificity of 82.5% for diagnosis of CFRLD according to our criteria and 46.5% sensitivity but 100% specificity in predicting an ultrasound/biopsy proven hepatic abnormality associated with CFRLD,” the researchers wrote. They discovered that the mAPRI was effective in differentiating between patients with and without CFRLD.

They concluded that the mAPRI is useful in the noninvasive diagnosis of CFRLD and provides insights into the potential clinical outcomes of patients depending on their current condition.

Reference

Khoshbin E, Clark S, Meachery G, et al. Diagnosis and predicted outcomes of patients with cystic fibrosis related liver disease considered for lung transplantationClin Transplant. Published online May 19, 2022. doi:10.1111/ctr.14713