Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study

Background/Aims: Liver disease is a leading cause of morbidity in adult pat ients with cystic fibrosis. Diagnosis of limited liver involvement in asymp tomatic patients is important since a safe and effective treatment with urs odeoxycholic acid can be used. We carried out a prospective open study to d escribe the intrahepatic biliary lesions using magnetic resonance cholangio graphy. Methods; Twenty;seven adult patients with cystic fibrosis were prospectivel y enrolled, whatever their hepatobiliary status. All patients underwent liv er function tests, ultrasonography and magnetic resonance cholangiography. Magnetic resonance cholangiograms were acquired on a Philips 1.5 Tesla unit using a 3D TSE MR sequence. Acquisition parameters (120 slices, 1,6 mm thi ckness, interslice overlap 0.8 mm) were followed by MIP reconstruction in t wo orthogonal planes. Magnetic resonance cholangiography images were assess ed for the presence of stenosis, dilatations and rigidity corresponding to current criteria of cholangitis. Among the 27 cystic fibrosis patients, 18 (Group I) fulfilled none of the clinical, biological or ultrasonographic cr iteria of liver disease;the remaining nine (Group II) fulfilled the criteri a for liver disease. In every patient, current causes of secondary sclerosi ng cholangitis had been excluded. Results All the Group II patients had abnormal magnetic resonance cholangio grams with features resembling those of primary sclerosing cholangitis in f ive, and simple biliary lesions in four. Nine Group L patients had abnormal magnetic resonance cholangiograms with primary sclerosing cholangitis-like lesions in five and simple biliary lesions in four. Magnetic resonance cho langiography anomalies were always dilatations, either isolated or associat ed with strictures and rigidity, both resembling those seen in cholangitis. They were seen in all the patients with known liver disease and in half th e patients without evidence of liver disease. Conclusion: This study confirms the high frequency of intrahepatic biliary abnormalities in CF patients, which is probably underestimated by clinical, biological and ultrasonographic evaluation. The magnetic resonance cholang iography technique could be useful to detect early intrahepatic biliary tra ct involvement in cystic fibrosis patients.