AI Article Synopsis

  • The clinical presentation of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome (OS) is similar, but treatment approaches differ.
  • A study of 34 pediatric patients revealed a female predominance in AIH and a higher percentage of black patients in PSC/OS.
  • Diagnosis requires a combination of clinical history, laboratory tests, imaging studies, and liver biopsy, as biopsies cannot be used alone for diagnosis but are essential for assessing severity and progression of the disease.

Article Abstract

Unlabelled: Clinical presentation and histopathology of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome (OS) are similar, but their management is different. We conducted a pediatric retrospective cross-sectional study of 34 patients with AIH and PSC. AIH had female predominance (74%) and was lower in PSC (45%). There was a trend toward higher frequency of blacks in PSC/OS (55%) compared to Caucasians (36%) and Hispanics (9%), but not race differences in AIH. Inflammatory bowel disease (IBD) was present in 75% of PSC/OS. Plasma cells were not specific for AIH (found in 42% of PSC). Concentric fibrosis was not reliable for PSC as was found in 46% of AIH.

Conclusion: A combination of clinical history, laboratory tests, imaging studies and liver biopsy are required to confirm and properly treat AIH and PSC. Liver biopsy should be used to grade severity and disease progression, but cannot be used alone to diagnose these conditions.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4086229PMC
http://dx.doi.org/10.3109/15513815.2014.898721DOI Listing

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