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

doi:10.1016/S0168-8278(99)80259-1

Journal of Hepatology

Volume 30, Issue 6, June 1999, Pages 1052-1056

https://doi.org/10.1016/S0168-8278(99)80259-1 Get rights and content

Abstract

Background/Aims: Liver disease is a leading cause of morbidity in adult patients with cystic fibrosis. Diagnosis of limited liver involvement in asymptomatic patients is important since a safe and effective treatment with ursodeoxycholic acid can be used. We carried out a prospective open study to describe the intrahepatic biliary lesions using magnetic resonance cholangiography.

Methods: Twenty-seven adult patients with cystic fibrosis were prospectively enrolled, whatever their hepatobiliary status. All patients underwent liver 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 thickness, interslice overlap 0.8 mm) were followed by MIP reconstruction in two orthogonal planes. Magnetic resonance cholangiography images were assessed 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 criteria of liver disease; the remaining nine (Group II) fulfilled the criteria for liver disease. In every patient, current causes of secondary sclerosing cholangitis had been excluded.

Results: All the Group II patients had abnormal magnetic resonance cholangiograms with features resembling those of primary sclerosing cholangitis in five, and simple biliary lesions in four. Nine Group I patients had abnormal magnetic resonance cholangiograms with primary sclerosing cholangitis-like lesions in five and simple biliary lesions in four. Magnetic resonance cholangiography anomalies were always dilatations, either isolated or associated with strictures and rigidity, both resembling those seen in cholangitis. They were seen in all the patients with knownliver disease and in half the 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 cholangiography technique could be useful to detect early intrahepatic biliary tract involvement in cystic fibrosis patients.

Section snippets

Patients

Twenty-seven adult CF patients (16 males and 11 females; mean age 27 (19–42)) referred to the Adult Cystic Fibrosis Center were prospectively included from May 1996 to May 1998, whatever their hepatobiliary status. Diagnosis of CF had been made by the association of typical symptoms of pulmonary or pancreatic disease together with at least two abnormal chloride concentrations on sweat testing. Body mass index was 20.1 (13.6–25); 23 patients had pancreatic insufficiency and four diabetes

Biological data and ultrasound findings

Nine of the 27 patients had clinical, morphological or biological evidence of liver involvement, defining the group II. One had clinical and ultrasound criteria of cirrhosis with a nodular appearance (16) and portal hypertension (esophageal varices, portal dilatation and splenomegaly). Three patients had clinical hepatomegaly with a heterogeneous appearance on ultrasonography, and repeated abnormal liver function tests (LFT). One patient had clinical hepatomegaly, normal LFT, and a homogeneous

Discussion

The 33% prevalence of clinical or biological involvement of the liver in our 27 patients is similar to published data in adult patients with CF 1., 2.. Intrahepatic biliary anomalies were shown in 18 out of 26 MRC studies (69%), very close to the prevalence of biopsy and autopsy studies in adult patients 4., 5.. All the patients with known liver disease had an abnormal appearance of their intrahepatic biliary tract, suggesting good sensitivity of the MRC, although the number of patients in this

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

Abstract

Section snippets

Patients

Biological data and ultrasound findings

Discussion

Hepatology

Lancet

Hepatology

Gastroenterology

J Hepatol

Hepatology

Gastroenterology

Treatment and prognosis of symptomatic gallbladder disease in patients with cystic fibrosis

J Pediatr Gastroenterol Nutr

Cystic fibrosis in adolescents and young adults

Thorax

Pathology of cystic fibrosis: review of the literature and comparison with 146 autopsied cases