Department of Pediatrics, Eastern Yokohama Hospital, Yokohama, Kanagawa, Japan.
J Pediatr Gastroenterol Nutr. 2011 Apr;52(4):470-3. doi: 10.1097/MPG.0b013e3181fc1e0b.
Although the diagnostic scoring system of autoimmune hepatitis (AIH) has been used, these criteria are intended mainly as research tools and are complicated to apply. To resolve these difficulties and allow quick diagnosis, a simplified scoring system was proposed in 2007. We retrospectively compared the simplified AIH scoring system with the 1999 revised original AIH scoring system in children.
Twenty children (boys/girls 10/10, age 1-15 years, mean age ± SD 8.4 ± 4.4 years) who were diagnosed with AIH based on clinical, biochemical, immunological, and histological data were enrolled in this study. In addition, 36 children with non-AIH liver diseases (boys/girls 22/14, age 1-16 years, mean age ± SD 7.8 ± 4.4 years) were available for evaluation of both the simplified and the 1999 revised scoring system.
The sensitivity and specificity of the 1999 revised scoring system were 100% and 81%, respectively. In contrast, the sensitivity and specificity of the simplified scoring system were 55% and 86%, respectively. Of the 20 children with AIH, 9 (45%) were classified as not having AIH using the simplified scoring system. Of the 9 children, 2 and 7 were classified as having definite AIH and probable AIH using the 1999 revised scoring system, respectively. All 5 children with primary sclerosing cholangitis were graded as having AIH using the simplified AIH criteria and the 1999 revised criteria.
Although the simplified AIH scoring system has low sensitivity for the diagnosis of AIH in children, the specificity of the simplified AIH scoring system is high. However, the simplified AIH scoring system could not differentiate between AIH and primary sclerosing cholangitis. Therefore, the simplified AIH scoring system does not seem to be a reliable diagnostic tool in children.
尽管已使用自身免疫性肝炎（AIH）的诊断评分系统，但这些标准主要用作研究工具，应用起来较为复杂。为了解决这些困难并实现快速诊断，2007 年提出了简化评分系统。我们回顾性地比较了简化 AIH 评分系统与儿童中 1999 年修订的原始 AIH 评分系统。
本研究纳入了 20 名基于临床、生化、免疫和组织学数据诊断为 AIH 的儿童（男/女 10/10，年龄 1-15 岁，平均年龄±标准差 8.4±4.4 岁）。此外，还纳入了 36 名患有非 AIH 肝病的儿童（男/女 22/14，年龄 1-16 岁，平均年龄±标准差 7.8±4.4 岁），用于评估简化和 1999 年修订评分系统。
1999 年修订评分系统的敏感性和特异性分别为 100%和 81%。相比之下，简化评分系统的敏感性和特异性分别为 55%和 86%。在 20 名 AIH 患儿中，有 9 名（45%）使用简化评分系统被归类为不具有 AIH。在这 9 名患儿中，有 2 名和 7 名分别使用 1999 年修订评分系统被归类为明确 AIH 和可能 AIH。所有 5 名原发性硬化性胆管炎患儿均使用简化 AIH 标准和 1999 年修订标准被归类为具有 AIH。
尽管简化 AIH 评分系统对儿童 AIH 的诊断敏感性较低，但简化 AIH 评分系统的特异性较高。然而，简化 AIH 评分系统无法区分 AIH 和原发性硬化性胆管炎。因此，简化 AIH 评分系统在儿童中似乎不是一种可靠的诊断工具。
