Autoimmune hepatitis and primary sclerosing cholangitis in children and adolescents

Claudia Patricia Rojas, Rajasekhar Bodicharla, German Campuzano-Zuluaga, Lina Hernandez, Maria Matilde Rodriguez

Research output: Contribution to journalArticlepeer-review

27 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)202-209
Number of pages8
JournalFetal and Pediatric Pathology
Issue number4
StatePublished - Aug 2014
Externally publishedYes


  • Autoantibodies
  • Cholestasis
  • Cirrhosis
  • Hepatitis
  • Liver biopsy
  • Pediatric

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Pediatrics, Perinatology, and Child Health


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