Autoimmune hepatitis in India: profile of an uncommon disease.
Autoimmune hepatitis in India: profile of an uncommon disease.
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DOI：
10.1186/1471-230x-5-27
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发表时间：
2005-08-15
影响因子：
2.4
通讯作者：
Alexander G
中科院分区：
文献类型：
--
作者：
Choudhuri G;Somani SK;Baba CS;Alexander G
据报道，自身免疫性肝炎（AIH）的发生率和临床表现在地理上存在相当大的差异。它被认为是印度肝脏疾病的罕见原因。本研究旨在确定世界这一地区AIH的发病率、临床、生化和组织学特征。对1999年1月至2002年6月期间出现急性或慢性肝病的患者进行前瞻性评价。AIH诊断采用国际自身免疫性肝炎组标准。检查包括临床、生化、USG、病毒标志物、UGI内镜检查、使用间接免疫荧光的AI标志物（ANA、SMA、抗LKM、AMA、RF、p-ANCA）和肝活检（如可能）。2401例患者中有41例（1.70%）被诊断为自身免疫性肝病。其中38例为自身免疫性肝炎，3例为原发性胆汁性肝硬化。自身免疫性肝炎患者的平均年龄为36.2（15.9）岁，34（89.4%）例为女性，症状持续时间为20.3（20.5）个月。慢性肝炎19例（50%），肝硬化13例（34.2%），急性肝炎5例（13.1%），淤胆型肝炎1例（2.6%）。黄疸21例（55.2%），足水肿、肝肿大17例（44.7%），脾肿大13例（34.2%），脑病、腹痛9例（23.6%），发热8例（21%）。12例食管静脉曲张，3例流血的。生化参数为ALT 187（360）U/L、AST 157（193）U/L、ALP 246（254）U/L、球蛋白4.1（1.6）g/dL、白蛋白2.8（0.9）g/dL、胆红素5.2（7.4）mg/dL、凝血酶原时间17（7）秒和ESR 47（17）秒。自身免疫性标志物SMA 24例，ANA 15例，SMA和ANA均4例，AMA 1例，类风湿因子2例，pANCA 1例，抗LKM 0例。30例（79%）患者有明确的AIH，8例（21%）患者有可能的AI肝炎。相关自身免疫性疾病15/38例（39.4%），糖尿病4例，甲状腺功能减退症3例，白癜风2例，血小板减少症2例，类风湿性关节炎2例，干燥综合征1例，自身免疫性多腺体综合征III型1例。2例患者病毒标志物阳性，1例表现为急性肝炎和HEV-IgM阳性，另1例抗-HCV阳性。在印度，自身免疫性肝炎并不常见，通常表现为慢性肝炎或肝硬化，急性肝炎不太常见。发病年龄较早，但临床参数和相关自身免疫性疾病与西方报道相似。原发性胆汁性肝硬化是罕见的。未观察到II型AIH。
Autoimmune hepatitis (AIH) has been reported to show considerable geographical variation in frequency and clinical manifestations. It is considered a rare cause of liver disease in India. The present study was undertaken to determine the incidence, clinical, biochemical and histological profile of AIH in this part of the world. Patients presenting with acute or chronic liver disease between January 1999 and June 2002 were evaluated prospectively. AIH was diagnosed using the international autoimmune hepatitis group criteria. Workup included clinical, biochemical, USG, viral markers, UGI endoscopy, AI markers (ANA, SMA, Anti-LKM, AMA, RF, p-ANCA) using indirect immunofluorescence and liver biopsy if possible. Forty-one of 2401 (1.70%) patients were diagnosed to have autoimmune liver disease. Out of these, 38 had autoimmune hepatitis and the rest 3 had primary biliary cirrhosis. The mean age of the patients of autoimmune hepatitis was 36.2 (15.9) years, 34 (89.4%) were females, and the duration of symptoms was 20.3 (20.5) months. Nineteen (50%) of them presented with chronic hepatitis, 13 (34.2%) as cirrhosis, 5 (13.1%) with acute hepatitis and 1 (2.6%) with cholestatic hepatitis. The presentations were jaundice in 21 (55.2%), pedal edema and hepatomegaly in 17 (44.7%), splenomegaly in 13 (34.2%), encephalopathy, abdominal pain in 9 (23.6%) and fever in 8 (21%). Twelve had esophageal varices and 3 had bled. Biochemical parameters were ALT 187 (360) U/L, AST 157 (193) U/L, ALP 246 (254) U/L, globulin 4.1 (1.6) g/dL, albumin 2.8 (0.9) g/dL, bilirubin 5.2 (7.4) mg/dL, prothrombin time 17 (7) sec and ESR 47 (17) sec. The autoimmune markers were SMA (24), ANA (15), both SMA and ANA (4), AMA (1), rheumatoid factor (2), pANCA (1), and Anti-LKM in none. Thirty (79%) patients had definite AIH and eight (21%) had probable AI hepatitis. Associated autoimmune diseases was seen in 15/38 (39.4%), diabetes 4, hypothyroidism 3, vitiligo 2, thrombocytopenia 2, rheumatoid arthritis 2, Sjogren's syndrome 1 and autoimmune polyglandular syndrome III in 1. Viral markers were positive in two patients, one presenting as acute hepatitis and HEV-IgM positive and another anti-HCV positive. In India, autoimmune hepatitis is uncommon and usually presents with chronic hepatitis or cirrhosis, acute hepatitis being less common. Age at presentation was earlier but clinical parameters and associated autoimmune diseases were similar to that reported from the west. Primary biliary cirrhosis is rare. Type II AIH was not observed.