Debbeche Radhouane, Maamouri Nadia, Ajmi Salem, Azzouz Mohamed Moussaddek, Ben Mami Nabyl, Dougui Mohamed Hédi, Filali Azza, Ghorbel Abdeljabbar, Khedhiri Faouzi, Krichene Mohamed Salah, Najjar Taoufik, Saffar Hammouda, Zouari Béchir
Service de Gastro-entérologie, Hôpital Charles Nicolle, Tunis.
Tunis Med. 2010 Nov;88(11):834-40.
Autoimmune hepatitis (AIH) is a chronic inflammatory condition of the liver of unknown etiology. Its epidemiological and anatomoclinical characteristics and its outcome were unknown in Tunisia.
To analyse epidemiological, anatomoclinical, immunological and histological aspects of AIH and to determine factors predicting relapse after treatment and death of this disease in Tunisia.
Patients presenting with AIH between January 1996 and December 2004 were evaluated in retrospective multicentric study. The diagnosis of AIH was established according to the criteria of the revised score of the international autoimmune hepatitis group (1999)
Eighty three patients were identified (70 female; mean age=49+17.9 years). 63% presented probable AIH and 37% presented definite AIH. Thirty two percent presented with the acute pattern. Eighty three per cent of cases were type I AIH and 5 % of cases were type II HAI. Fifty seven percent of the patients were cirrhotic at presentation. Associated autoimmune diseases was seen in 27 patients, dominated by diabetes, autoimmune thyroiditis and Sjögren's syndrome. An overlap syndrome was diagnosed in 25% of cases; primary biliary Cirrhosis-AIH in 20% of cases and primary sclerosing cholangitis-AIH in 5% of cases. Fifty patients were treated by glucocorticoids as monotherapy or in combination with azathioprine. Complete remission was achieved in 90% of cases. Fourteen percent relapsed within a median time of 12 months. Factors associated with relapse were: treatment with Azathioprine<18 months, absence of lobular necrosis and anti-nuclear antibody (+) profile. Mortality was observed in 17 % of cases. Factors associated with death were encephalopathy as an independent factor and treatment with Azathioprine<18 months.
In Tunisia, epidemiological and clinical characteristics of AIH were similar to those reported in the literature but with a higher frequency of cirrhosis at presentation. Treatment with Azathioprine < 18 months was the main factor associated with relapse and represented with encephalopathy a factor associated with death.
自身免疫性肝炎（AIH）是一种病因不明的肝脏慢性炎症性疾病。其流行病学、解剖临床特征及预后在突尼斯尚不清楚。
分析突尼斯AIH的流行病学、解剖临床、免疫和组织学特征，并确定预测该疾病治疗后复发及死亡的因素。
对1996年1月至2004年12月期间诊断为AIH的患者进行回顾性多中心研究。AIH的诊断依据国际自身免疫性肝炎小组（1999年）修订评分标准确定。
共纳入83例患者（70例女性；平均年龄=49±17.9岁）。63%为疑似AIH，37%为确诊AIH。32%表现为急性型。83%的病例为Ⅰ型AIH，5%的病例为Ⅱ型AIH。57%的患者初诊时已出现肝硬化。27例患者伴有自身免疫性疾病，以糖尿病、自身免疫性甲状腺炎和干燥综合征为主。25%的病例诊断为重叠综合征；20%的病例为原发性胆汁性肝硬化-AIH，5%的病例为原发性硬化性胆管炎-AIH。50例患者接受了糖皮质激素单药治疗或联合硫唑嘌呤治疗。90%的病例实现完全缓解。14%的患者在中位时间12个月内复发。与复发相关的因素有：硫唑嘌呤治疗时间<18个月、无小叶坏死和抗核抗体（+）谱。17%的病例观察到死亡。与死亡相关的因素有：脑病作为独立因素以及硫唑嘌呤治疗时间<18个月。
在突尼斯，AIH的流行病学和临床特征与文献报道相似，但初诊时肝硬化的发生率更高。硫唑嘌呤治疗时间<18个月是与复发相关的主要因素，而脑病是与死亡相关的因素。
