Characteristics of autoimmune hepatitis in Taiwan: the 11 years' experiences of a medical center.

Hui-Chun Huang, Yi-Shin Huang, Jaw-Ching Wu, Shyh-Haw Tsay, Teh-Ia Huo, Yuan-Jen Wang, Jing-Chuan Lo, Chih-Yen Chen, Chun-Ping Li, Full-Young Chang, Shou-Dong Lee
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Abstract

Background: Unlike in Western countries, autoimmune hepatitis (AIH) is an infrequent diagnosis in Taiwan. The clinical characteristics of AIH in this area are unclear. The aim of this study was to elucidate the clinical features of AIH in Taiwan.

Methods: All the medical records of in-patients with the diagnosis of chronic hepatitis in our hospital from 1990 to 2001 were reviewed for the possibility of AIH. The clinical features, biochemical data, immunological presentations, treatments and survival of the patients were evaluated.

Results: Twenty-two patients (15 females and 7 males) were diagnosed as having AIH within 11 years. The median age at onset was 64 years (range: 17-77 years). Compared with female patients, male patients had older age (p = 0.001), shorter duration from initial presentation of symptoms to diagnosis (p = 0.015), lower serum levels of alkaline phosphatase (ALK-P, p = 0.022) and albumin (p = 0.043). Five (23%) patients presented with cirrhosis upon diagnosis. Compared with non-cirrhotic patients, cirrhotic patients had lower serum levels of alanine aminotransferase (p = 0.002), aspartate aminotransferase (p = 0.015), gamma-glutamyl transferase (G-GT, p = 0.002), albumin (p = 0.14), white cell counts (p = 0.009) and platelet counts (p = 0.002). Thirteen (59%) patients had concomitant clinico-pathological features of cholestatic liver disease (ALK-P > or = 2 times of upper normal limit or pathologic evidence of cholangiopathy). They had higher serum levels of ALK-P (p < 0.001) and G-GT (p = 0.004) than 9 non-cholestatic patients. There were no significant differences in survival between these groups. The prescribed initial and maintained prednisolone dosages for our patients to control disease activity were 19 +/- 15 mg and 8 +/- 1 mg, respectively, which were lower than those recommended in Western countries. The remission rate to steroid treatment and relapse rate after discontinuing corticosteroids were 87.5% and 50%, respectively.

Conclusions: Compared with Western AIH patients, the AIH patients in Taiwan are older and more likely to develop cholestasis, and need a relatively lower dose of steroid for treatment. Owing to one quarter of the patients already having liver cirrhosis on diagnosis, AIH should be suspected in any Taiwanese patient with cryptogenic hepatitis or cirrhosis.

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台湾自身免疫性肝炎的特点:某医疗中心11年的经验。
背景:与西方国家不同,自身免疫性肝炎(AIH)在台湾并不常见。该地区AIH的临床特征尚不清楚。摘要本研究旨在探讨台湾地区AIH的临床特征。方法:对我院1990 ~ 2001年诊断为慢性肝炎的住院患者的病历资料进行回顾性分析,探讨其感染的可能性。对患者的临床特征、生化指标、免疫学表现、治疗及生存期进行评价。结果:11年内确诊为AIH的患者22例,其中女性15例,男性7例。中位发病年龄为64岁(范围:17-77岁)。与女性患者相比,男性患者年龄较大(p = 0.001),从最初出现症状到诊断的时间较短(p = 0.015),血清碱性磷酸酶(ALK-P, p = 0.022)和白蛋白水平较低(p = 0.043)。5例(23%)患者在诊断时出现肝硬化。与非肝硬化患者相比,肝硬化患者血清丙氨酸转氨酶(p = 0.002)、天冬氨酸转氨酶(p = 0.015)、γ -谷氨酰转移酶(G-GT, p = 0.002)、白蛋白(p = 0.14)、白细胞计数(p = 0.009)和血小板计数(p = 0.002)水平均较低。13例(59%)伴有胆汁淤积性肝病的临床病理特征(ALK-P >或=正常上限的2倍或有胆管病的病理证据)。血清ALK-P (p < 0.001)和G-GT (p = 0.004)水平高于9例非胆汁淤积症患者。两组患者的生存率无显著差异。我们的患者用于控制疾病活动性的处方初始和维持泼尼松龙剂量分别为19 +/- 15mg和8 +/- 1mg,低于西方国家的推荐剂量。类固醇治疗缓解率为87.5%,停用皮质类固醇后复发率为50%。结论:与西方AIH患者相比,台湾AIH患者年龄较大,更容易发生胆汁淤积,需要相对较低剂量的类固醇治疗。由于四分之一的患者在诊断时已经有肝硬化,任何台湾的隐源性肝炎或肝硬化患者都应该怀疑AIH。
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Hereditary hemorrhagic telangiectasia. Juvenile myoclonic epilepsy. Congenital muscular dystrophy. Autoimmune hepatitis: truly a rare disorder in Taiwan. Characteristics of autoimmune hepatitis in Taiwan: the 11 years' experiences of a medical center.
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