Overlap syndrome of autoimmune chronic liver diseases: MRI findings

To describe the MR findings of overlap syndromes of autoimmune chronic liver diseases.

Review of clinical and radiological databases between March 2001 and July 2008 for patients with a clinical diagnosis and liver biopsy features compatible with overlap syndrome who had also undergone an abdominal MRI yielded 15 adult patients. MR features of overlap syndrome were reviewed by two radiologists by consensus. Two radiologists independently reviewed the studies in a blinded fashion for primary sclerosing cholangitis (PSC)-type or non-PSC-type features to assess interobserver agreement.

PSC-type and non-PSC-type overlap syndrome had distinctive MR features. In the consensus review, all 12 patients with PSC-type overlap syndrome were correctly identified. Imaging findings included central macroregeneration (N = 9), peripheral atrophy (N = 7), biliary duct beading (N = 12), biliary dilation (N = 10), or a combination of all four findings (N = 7). The presence of any of the first three features had 100% specificity for a PSC-type overlap syndrome. Patients with non-PSC-type overlap (N = 3) showed relatively normal liver morphology (N = 3) and minimal biliary ductal dilatation in one patient. There was good interobserver agreement (kappa = 0.76).

Patients with serologic or pathologic evidence of AIH or PBC with imaging features of PSC may have PSC-type overlap syndrome.