TIMP-1/-2 and transient elastography allow non invasive diagnosis of cystic fibrosis associated liver disease

BackgroundCystic fibrosis (CF) associated liver disease develops in approximately 30% of CF patients. However, routine sensitive diagnostic tools are lacking.AimsWe aimed to compare the value of transient elastography and experimental fibrosis markers for the detection of liver disease in CF patients.Methods145 CF patients (75 children, 70 adults) were prospectively studied and received transient elastography. CF liver disease was diagnosed according to recent guidelines. Serum concentrations of YKL-40, HA, PIIIP, MMP-9, TIMP-1 and TIMP-2 were determined by ELISA.ResultsTransient elastography was increased in adults and children with CF hepatopathy compared to those without and exhibited a high diagnostic accuracy for CF liver disease. In adults with portal hypertension, elastography was further enhanced. TIMP-2 was elevated in adults with CF hepatopathy associated portal hypertension and exhibited a high diagnostic accuracy for portal hypertension in adults and for CF hepatopathy in children. TIMP-1 had a high diagnostic accuracy for CF hepatopathy in adults. Diagnostic sensitivities were increased when elastography and respective biomarkers were combined for the detection of CF hepatopathy and portal hypertension.ConclusionsTIMP-1 and TIMP-2 represent powerful biomarkers for CF associated liver disease and portal hypertension. Their determination may confirm and improve the diagnostic accuracy of transient elastography.