Rojas Claudia Patricia, Bodicharla Rajasekhar, Campuzano-Zuluaga German, Hernandez Lina, Rodriguez Maria Matilde
1Department of Pathology, University of Miami, Holtz Children's Hospital, Miami, FL, USA.
Fetal Pediatr Pathol. 2014 Aug;33(4):202-9. doi: 10.3109/15513815.2014.898721. Epub 2014 Apr 22.
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.
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.
自身免疫性肝炎（AIH）与原发性硬化性胆管炎（PSC）重叠综合征（OS）的临床表现和组织病理学相似，但治疗方法不同。我们对34例AIH和PSC患者进行了一项儿科回顾性横断面研究。AIH以女性为主（74%），而PSC中女性比例较低（45%）。与白种人（36%）和西班牙裔（9%）相比，PSC/OS中黑人的比例有更高的趋势（55%），但AIH中不存在种族差异。75%的PSC/OS患者存在炎症性肠病（IBD）。浆细胞并非AIH所特有（在42%的PSC中也有发现）。同心圆状纤维化对PSC来说并不可靠，因为在46%的AIH中也有发现。
需要结合临床病史、实验室检查、影像学研究和肝活检来确诊并正确治疗AIH和PSC。肝活检应用于评估疾病严重程度和进展，但不能单独用于诊断这些疾病。
