青少年自身免疫性肝病患者的临床观察

Clinical observation of adolescent patients with autoimmune liver disease

目的分析青少年自身免疫性肝病患者的临床病理学特征及治疗转归,提高对该病的认识。方法回顾性分析2016年9月至2019年10月于上海交通大学医学院附属仁济医院消化科住院及门诊随访的32例青少年自身免疫性肝病患者的分型、临床特点、生物化学指标、肝脏病理、治疗效果及药物不良反应。结果32例患者中自身免疫性肝炎(AIH)28例(1型AIH 25例,2型AIH 3例),自身免疫性硬化性胆管炎(ASC)4例。临床表现为急性肝炎型起病7例(21.9%),慢性隐匿型起病22例(68.8%),肝硬化及其并发症型起病3例(9.4%)。有13例患者单用激素治疗,2例因治疗时间未满1年故疗效是否应答不确切,1例不完全应答,10例完全应答,其中1例已成功停药,2例在完全应答缓解数年后复发,在激素基础上加用免疫抑制剂(这2例归为联合治疗)。21例根据病情应用激素联合免疫抑制剂治疗,11例不完全应答,10例完全应答。结论青少年AIH的临床表现多样,诊断困难,但对激素单药或联合硫唑嘌呤的免疫抑制治疗反应良好。AIH可转归至ASC,应定期对AIH患儿行磁共振胰胆管造影(MRCP)或逆行胰胆管造影(ERCP)检查以排查ASC,尤其是出现胆汁淤积症状者。

Objective This paper attempts to review and analyze the clinicopathological characteristics and treatment outcomes of adolescents with autoimmune liver disease to improve their understanding of the disease.Methods A retrospective analysis was performed for the clinical manifestation,biochemical examinations,liver pathology results,outcomes of treatment and drug side effects,and follow-up data of 32 adolescents with autoimmune liver diseases who were treated in the department of gastroenterology,Renji Hospital Affiliated to the School of Medicine,Shanghai Jiao Tong University from September 2016 to October 2019.Results Of the 32 patients,28 were autoimmune hepatitis(AIH)cases(25 type 1 AIH cases and 3 type 2 AIH cases)and 4 were autoimmune sclerosing cholangitis(ASC)cases.In clinical manifestations,there were 7 cases(21.9%)of acute hepatitis,22 cases(68.8%)of hidden onset,and 3 cases(9.4%)of cirrhosis and complications.Of the 13 patients who received prednisolone monotherapy,2 were treated for less than 1 year.Partial remission was seen in 1 patient,complete remission in 10 patients.Of the complete remission patients,1 discontinued treatment successfully,and 2 relapsed after complete remission so that immunosuppressant was added on the basis of prednisolone.According to the severity of the disease,21 patients were treated with prednisolone combined immunosuppressive therapy.Partial remission was seen in 11 patients,complete remission in 10 patients.Conclusions The clinical manifestations of AIH in adolescent are diverse and difficult to diagnose,but respond well to hormone monotherapy or immunosuppressive therapy combined with azathioprine.AIH can develop to ASC,and adolescent with AIH should be regularly examined for ASC by performing magnetic resonance cholangiopancreatography(MRCP)or retrograde cholangiopancreatography(ERCP)in every child with AIH,especially those with symptoms cholestasis.