摘要
自體免疫性肝炎(Autoimmune hepatitis)(AIH)是一種少見的肝臟慢性炎症，尤其在台灣更為罕見，之前國內文獻記載病例數目不多。AIH的診斷不易，因為缺乏簡單及有效的診斷方法，自從International Autoimmune Hepatitis Group(IAIHG)於1993年推薦AIH的診斷評分制度(diagnostic criteria and scoring system)，復於1999年經過修正後，診斷AIH變為更有效及平易，奇美醫院憑藉此評分制度於最近三年診斷了27例的AIH病例，今提出此臨床研究報告。自2000年到2002年9月，共收集27例診斷為AIH病，並對病人之詳細病史（包括疾病表現、B型及C型肝炎、酒精、藥物以及家族史），臨床症狀，類固醇治療，預後以及存活率進行詳盡的調查及分析。憑藉IAIHG的評分制度，15分以上歸類為Definite AIH，10-15分歸類為Probable AIH，兩組病人都列入此研究。其中又對有肝硬化及無肝硬化組進行比較分析。病人臨床數據皆用平均值及中位數表示，統計方法採用Fisher's exact test，Wilcoxon Rank Sum test及Kaplan-Meier curve。27位病人被診斷為AIH type l，Definite AIH 13人，Probable AIH 14人，平均年齡為58歲，男女比例為4:23。63%病人以漸進性發作表現，37%病人為急性發作，最常見的臨床症狀依序為倦怠(56%)、黃疸(52%)、及腹脹(52%)。96%病人ANA(antinuclear antibodies)positive，檢驗結果異常依序為AST(93%)、ALT(93%)、serum globulin(77%)，bilirubin(71%)，病人呈現嚴重肝功能異常者(bilirubin增加，albumin降低，prothrombin time延長）比例偏高。37%的AIH病人呈現肝硬化，與非肝硬化組比較，統計顯示prolonged prothrombin time(17.3s vs 12.2s，P=0.030)，poorer outcome(P=0.041)及ANA titer偏高（480倍vs 80倍P=0.035）為有意義差別。13位病人接受肝組織切片檢查，最常見病理表現為Interface hepatitis (100%)及Lymphoplasmacytic cells infiltration (100%)。部份病人接受類固醇治療，反應良好。值得一提為肝硬化及非肝硬化病人對類固醇治療反應並無多大差異。整體存活率為88.9%。本研究顯示自體免疫性肝炎(AIH)在台灣的發生率比以往所認知的高很多。絕大多數病人屬於AIH type l。雖然疾病呈現期肝機能損害偏於嚴重，總體而言，此類病人的預後尚佳。
關鍵字
無資料並列摘要
Autoimmune hepatitis (AIH) is thought to be rare in Taiwan. By using the diagnostic scoring system recommended by the International Autoimmune Hepatitis Group, 27 cases of AIH patients had been diagnosed. We herein describe the study with respect to the clinical, laboratory and histological features at presentation. All patients were consecutively diagnosed over a period of 3 years. The medical records of patients were reviewed and analyzed. The clinical information, laboratory data and histological findings were assessed in great detail. Twenty-seven patients were diagnosed as AIH Type I, with a median age of 58 years and a female-male ratio of 23:4. The most common clinical features at presentation were fatigue, jaundice and abdominal fullness. ANA positive rate was 96%, and most of the patients showed elevated values of AST, ALT, serum globulin and bilirubin. A substantial proportion of patients presented with poor liver function at entry and 37% of patients had liver cirrhosis, with relatively prolonged PT (p=0.030) and poorer outcome (p=0.041) as compared to the non-cirrhotics. As a whole there was a favorable treatment response and the overall survival rate is 88.9%. The AIH incidence is much higher than previously presumed and AIH type I is the predominant type of the disease. Although a substantial proportion of AIH patients presented with poor hepatic function at entry, the overall survival rate is high (88.9%).
延伸閱讀
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