Objective: To review and analyze the clinical and pathological data of children with autoimmune hepatitis (AIH). Methods: Medical records of 46 patients hospitalized in Pediatric Liver Diseases Treatment and Research Center, Fifth Medical Center, General Hospital of People's Liberation Army(PLA) from April 2012 to April 2018 were extracted. Medical data included type of AIH, clinical manifestations, biochemical parameters, liver biopsy results, and outcomes of treatment were analyzed retrospectively. Among 46 children, 19 were males and 27 were females. The age of onset was 10.1(1.4-18.0) years old. Chi-Square test, Rank sum test or t test were used for inter-group comparison. Results: There were 32 (70%)AIH-I cases and 14 (30%)AIH-Ⅱ cases (χ(2)=12.565, P=0.000). Among the 46 patients, there were 5 modes of onest: 17 cases (37%) had acute viral hepatitis-like presentation, 2 cases (4%) had fulminant hepatic failure, 9 cases (20%) had insidious onset, 5 cases (11%) showed cirrhosis and portal hypertension, and 13 cases (28%) were incidentally found to be due to elevated hepatic aminotransferases. Comorbidities including primary sclerotic cholangitis (n=3), primary biliary cholangitis (n=1), systemic lupus erythematosus (n=1) and inflammatory bowel disease (n=2), were all seen in AIH-Ⅰ cases. The elevated biochemical parameters of these patients were as follows: alanine aminotransferase (n=46), aspartate transminase (n=46), total bilirubin (n=35) γ-glutamyl transpeptadase (n=39), γ-globulin (n=32) and IgG (n=33). The γ-globulin and IgG levels were significantly higher in AIH-Ⅰ patients than those with AIH-Ⅱ((32±9)% vs. (23±8)%, t=3.217, P=0.002,(27±10) vs. (18±8)g/L, t=3.193, P=0.003, respectively). Thirty-nine patients received liver biopsy, among whom 22 (56%) with inflammation grade (G)≥3, 26(67%) with fibrosis stage (S) ≥3, and 7 with hepatic cirrhosis (S4) according to pathological analysis. Typical histopathological changes of AIH included: 36 cases of interfacial hepatitis (92%), 23 cases of lymphocyte/plasma cell infiltration (59%), 3 cases of rosette (8%). Forty patients received prednisolone monotherapy or combined with azathioprine after diagnosis. Complete remission was seen in 29 (72%) patients, partial remission in 10 (25%) patients and no response in 1 (3%) patient. Among complete remission patients, 15 (52%) had relapse in the process of prednisolone reduction. Repeated liver biopsy performed in 8 patients after treatment showed that hepatic inflammation and fibrosis were both improved in 6 patients, only inflammation was alleviated without fibrosis improvement in 1 patient, and neither inflammation nor fibrosis was improved in 1 case. The length of follow-up was 3.3 (0.3-10.5) years, and none of the 39 prednisolone-responded cases discontinued treatment successfully. Adverse effect of long-term prednisolone therapy included bilateral cataract (n=6), spinal fracture accompanied with delayed bone age development (n=1). Conclusions: AIH-Ⅰ is more common than AIH-Ⅱ in children, with diverse clinical characteristics. Most cases have progressive liver inflammation and fibrosis when diagnosed. Prednisolone monotherapy or combined with azathioprine could achieve both biochemical and pathological improvement, but relapse is inevitable during drug tapering, hence long-term treatment is essential.
目的： 总结分析儿童自身免疫性肝炎（AIH）的临床病理特点及疗效，提高对该病的认识。 方法： 回顾性分析2012年4月至2018年4月在解放军总医院第五医学中心青少年肝病诊疗与研究中心住院的46例AIH患儿[男19例，女27例，发病年龄10.1（1.4~18.0）岁]的分型、临床特点、生化指标、肝脏病理、治疗效果，组间比较采用t检验、秩和检验或χ(2)检验。 结果： 46例患儿中AIH-Ⅰ型32例（70%），AIH-Ⅱ型14例（30%），差异有统计学意义（χ(2)=12.565，P=0.000）。分5种起病方式：类似急性病毒性肝炎样表现17例（37%），暴发性肝衰竭2例（4%），隐匿性发作9例（20%），合并肝硬化和门脉高压5例（11%），转氨酶升高（偶然化验发现）13例（28%）。AIH-Ⅰ型患儿中合并原发性硬化性胆管炎3例、原发性胆汁性胆管炎1例、炎症性肠病2例、系统性红斑狼疮1例。46例AIH患儿丙氨酸转氨酶（ALT）升高46例，天冬氨酸转氨酶（AST）升高46例，总胆红素（TBIL）升高35例，γ-谷氨酰基转移酶（GGT）升高39例，γ球蛋白升高32例，免疫球蛋白G（IgG）升高33例。AIH-Ⅰ型患儿γ球蛋白、IgG[（32±9）%、（27±10）g/L]均明显高于AIH-Ⅱ型患儿[（23±8）%、（18±8）g/L]，差异均有统计学意义（t=3.217、3.193，P=0.002、0.003）。39例患儿行肝脏病理检查，炎症分级G≥3的22例（56%）；纤维化分期S≥3的26例（67%），其中肝硬化7例。AIH特征性肝组织病理：可见界面性肝炎36例（92%），淋巴细胞或浆细胞浸润23例（59%），玫瑰花结3例（8%）。40例在诊断后接受单用激素（31例）或激素联合免疫抑制剂（9例）治疗，完全缓解29例（72%），部分缓解10例（25%），无应答1例（3%）。达到完全缓解后激素减量过程中出现复发15例（52%）。8例治疗前后均行肝脏穿刺，6例治疗后肝脏炎症及纤维化程度明显改善，1例炎症减轻，纤维化改善不明显，1例炎症与纤维化均无改善。随访时间3.3（0.3~10.5）年，39例激素应答的患儿无成功停药病例。长期激素治疗的不良反应：双眼白内障6例，1例伴脊柱骨折并骨龄发育落后。 结论： 儿童AIH-Ⅰ型明显多于Ⅱ型，临床表现多样化，比成人严重，大部分患儿发病时肝脏炎症和纤维化已显著进展。激素单药或联合硫唑嘌呤治疗后生化和病理可明显改善，但药物减量过程中易复发，需长期治疗。.
Keywords: Child; Diagnosis; Hepatitis, autoimmune; Pathology.