High frequency of liver abnormalities in adult cystic fibrosis patients after lung transplantation

Abstract

Background: CF associated liver disease (CFLD) mainly develops in early childhood and occurs in up to 30% of CF patients including features of steatosis, fibrosis and cholestasis. We aimed at characterizing liver disease in adult patients with CF after lung transplantation (LuTx). Methods: Consecutive patients admitted to our outpatient clinic received ultrasound, fibroscan, MRI/MRCP as well as testing of liver function tests, lipids and vitamin D. Liver diseases of other origins were excluded. Results: 36 patients (median age 30 y, IQR 25 – 38 y; 42% male) were included in the study. Median time since LuTx was 4.5 y (IQR: 2.3 – 8.3 y). Elevated AP/GGT were found in 33%, elevated ALT/AST levels in 28% of patients. In 43% (12/23) of patients steatosis and in 18% (4/23) either fibrosis or liver cirrhosis was diagnosed by ultrasound. In 39% (7/18) of patients MRI/MRCP revealed cholangiectasia, and delayed excretion of contrast medium in 20% (3/15). Fibroscan proved cirrhosis in 2 patients, whereas in all other patients fibrosis 0 to 1 was detected. No association of liver function tests and results of imaging was found. Steatosis was significantly associated with hypertriglyceridemia (p < 0.05), but not with hypercholesterolemia. Vitamin D levels in general were low. However, neither an association of vitamin D, nor gender or BMI with liver disease was evident. Conclusion: About 40% of post transplant patients show bile duct alterations suggesting presence of CFLD. Hepatic steatosis associated with hypertriglyceridemia is a common finding in adult CF patients after LuTx. Liver function tests seem to be a weak parameter for monitoring liver disease in CF patients.