Diagnosis and Predicted Outcomes of Patients with Cystic Fibrosis Related Liver Disease Considered for Lung Transplantation

Purpose Anxiety associated with poor post operative outcomes has led to patients with Cystic Fibrosis Related Liver Disease (CFRLD) being denied a chance of lung transplant for fear of developing post-operative progressive hepatic impairment. Methods We studied a total of 238 transplanted patients. Patients were divided into two groups CFRLD and Non-CFRLD based on our new criterion. Groups were compared first to assess validity of the diagnosis and then survival outcomes. A predictor of hepatic injury was devised by modifying the APRI (Aspartate aminotransferase-to-Platelet Ratio Index) scores to diagnose CFRLD and predict severity of liver disease. Results The new diagnostic criteria for differentiating CFRLD from Non CFRLD was effective. This was supported by significant difference in serum Bilirubin from 6.1 to 9.4 micromole / L at 90 days post transplant (p = 0.0001). There was no significant difference in the survival between the two groups at short, medium or long term demonstrated by the Kaplan-Meier plot with survival of 85%, 73%, 47%, 18.6% and 4.7% at one, two, five, ten and 15 year respectively. A modified APRI score of greater than 0.2 had a sensitivity of 43.0% but a specificity of 82.5 % for predicting CFRLD and 46.5% sensitivity but 100% specificity in diagnosing an ultrasound/biopsy proven hepatic abnormality associated with CFRLD. Conclusion Patients with CFRLD and portal hypertension but with preserved hepatocellular function have a similar outcome to patients without CFRLD. Modified APRI sore is a highly specific non-invasive tool for diagnosis of CFRLD.