TY - JOUR
T1 - Autoimmune hepatitis and primary sclerosing cholangitis in children and adolescents
AU - Rojas, Claudia Patricia
AU - Bodicharla, Rajasekhar
AU - Campuzano-Zuluaga, German
AU - Hernandez, Lina
AU - Rodriguez, Maria Matilde
PY - 2014/8
Y1 - 2014/8
N2 - 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.
AB - 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.
KW - Autoantibodies
KW - Cholestasis
KW - Cirrhosis
KW - Hepatitis
KW - Liver biopsy
KW - Pediatric
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UR - http://www.scopus.com/inward/citedby.url?scp=84903217371&partnerID=8YFLogxK
U2 - 10.3109/15513815.2014.898721
DO - 10.3109/15513815.2014.898721
M3 - Article
C2 - 24754367
AN - SCOPUS:84903217371
VL - 33
SP - 202
EP - 209
JO - Pediatric Pathology and Molecular Medicine
JF - Pediatric Pathology and Molecular Medicine
SN - 1551-3815
IS - 4
ER -
