Patients with PBC who fail to show a biochemical response to UDCA or who have ductopenia on baseline biopsy progress histologically during extended follow-up. Such patients may benefit from novel treatments, with our exploratory data providing a means of identifying these individuals early in their disease.

Secondary sclerosing cholangitis (SSC) is a disease that is morphologically similar to primary sclerosing cholangitis (PSC) but that originates from a known pathological process. Its clinical and cholangiographic features may mimic PSC, yet its natural history may be more favorable if recognition is prompt and appropriate therapy is introduced. Thus, the diagnosis of PSC requires the exclusion of secondary causes of sclerosing cholangitis and recognition of associated conditions that may potentially imitate its classic cholangiographic features. Well-described causes of SSC include intraductal stone disease, surgical or blunt abdominal trauma, intra-arterial chemotherapy, and recurrent pancreatitis. However, a wide variety of other associations have been reported recently, including autoimmune pancreatitis, portal biliopathy, eosinophillic and/or mast cell cholangitis, hepatic inflammatory pseudotumor, recurrent pyogenic cholangitis, primary immune deficiency, and AIDS-related cholangiopathy.

Large bile duct injury (that seen on cholangiography) is not usually considered a feature of autoimmune hepatitis (AIH) in adults but is present in up to 50% of children with AIH. The aim of this work was to study the prevalence of large bile duct abnormalities identified by magnetic resonance cholangiography (MRC) in adults given a diagnosis of AIH. Seventy-nine (n ‫؍‬ 79) patients given a diagnosis of AIH (mean AIH score: 15.1 ؎ 3.4) were screened with MRC for evidence of sclerosing cholangitis (SC). Results were reviewed by two radiologists. Clinical parameters were correlated with MRC findings. A histological review of available liver biopsies (n ‫؍‬ 29) was performed. Of the 79 patients surveyed, 8 (10%) had MRC findings consistent with primary sclerosing cholangitis (PSC). The interrater variability was excellent (kappa ‫؍‬ 0.87). Younger age at diagnosis (24.3 ؎ 11.9), higher baseline alkaline phosphatase (186.4 ؎ 98.3), higher bilirubin at time of MRC (45.8 ؎ 37.2), and greater lobular activity on initial liver biopsy were significantly associated with the detection of this overlap of SC with AIH (P ‫؍‬ 0.024, P ‫؍‬ 0.037, P ‫؍‬ 0.032, and P ‫؍‬ 0.041, respectively), but not alkaline phosphatase/aspartate aminotransferase ratio, time between the initial diagnosis of AIH and the MRC, or the presence of cirrhosis on initial liver histology. Two cases with a normal MRC had histological lesions typical of small duct PSC. Conclusion: The presence of SC detected by MRC and from liver histology in adult patients with AIH may not be clinically overt, and thus the prevalence of this AIH/SC overlap may be higher than previously recognized. Our data suggest that routine radiological evaluation of the biliary tree should be performed in adults given a diagnosis of AIH, as in children the presence of this overlap negatively impacts on survival. (HEPATOLOGY 2008;47:949-957.)