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

2012 Digestive & Liver Disease 44;9 (780-787)

BACKGROUND: Cystic fibrosis (CF) associated liver disease develops in approximately 30% of CF patients. However, routine sensitive diagnostic tools are lacking. AIMS: We aimed to compare the value of transient elastography and experimental fibrosis markers for the detection of liver disease in CF patients. METHODS: 145 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. RESULTS: Transient 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. CONCLUSIONS: TIMP-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.

Pubmed : 22652148