Frequency and nature of the variant syndromes of autoimmune liver disease
Frequency and nature of the variant syndromes of autoimmune liver disease
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DOI：
10.1002/hep.510280210
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发表时间：
1998-08-01
期刊：
影响因子：
13.5
通讯作者：
Czaja, AJ
中科院分区：
文献类型：
--
作者：
Czaja, AJ
为了确定自身免疫性肝病中变异综合征的频率和性质，对162例1型自身免疫性肝炎患者、37例原发性胆汁性肝硬化患者和26例原发性硬化性胆管炎患者进行了统一的评估，并使用评分系统对原始诊断的强度进行了评估。变体形式，包括自身免疫性肝炎和原发性胆汁性肝硬化综合征（7%）或原发性硬化性胆管炎（6%）和自身免疫性胆管炎（11%），在225例患者（18%）中很常见。在皮质类固醇治疗期间，自身免疫性肝炎和原发性胆汁性肝硬化患者与明确的自身免疫性肝炎患者一样普遍进入缓解期（75% vs. 64%, P = 0.5）。其疗效优于自身免疫性肝炎和原发性硬化性胆管炎（75% vs. 22%, P = 0.03）或自身免疫性胆管炎（75% vs. 0%, P = 0.009），自身免疫性肝炎和原发性硬化性胆管炎患者预后不良的频率高于其他变异（78% vs. 17%, P = 0.04），且其死于肝功能衰竭或需要肝移植的频率高于明确自身免疫性肝炎患者（33% vs. 8%, P = 0.05）。所有进入缓解期的患者血清碱性磷酸酶水平均未超过参考值的2倍。综上所述，自身免疫性肝病的变异是常见的。皮质类固醇治疗对具有自身免疫性肝炎和原发性胆汁性肝硬化特征的患者有效，血清碱性磷酸酶水平低于参考值的两倍是皮质类固醇反应性个体的特征。
To determine the frequency and nature of variant syndromes in autoimmune liver disease, 162 patients with type 1 autoimmune hepatitis, 37 patients with primary biliary cirrhosis, and 26 patients with primary sclerosing cholangitis were assessed in a uniform fashion, and the strength of the original diagnosis was evaluated by use of a scoring system. Variant forms, including syndromes with autoimmune hepatitis and primary biliary cirrhosis (7%) or primary sclerosing cholangitis (6%) and autoimmune cholangitis (11%), were common in the 225 patients (18%), Individuals with autoimmune hepatitis and primary biliary cirrhosis entered remission during corticosteroid therapy as commonly as individuals with definite autoimmune hepatitis (75% vs. 64%, P = .5), and they responded better than patients with autoimmune hepatitis and primary sclerosing cholangitis (75% vs. 22%, P = .03) or autoimmune cholangitis (75% vs. 0%, P = .009), Patients with autoimmune hepatitis and primary sclerosing cholangitis had a higher frequency of a poor result than other variants (78% vs. 17%, P = .04), and they died of liver failure or required liver transplantation more often than patients with definite autoimmune hepatitis (33% vs. 8%, P = .05). None of the patients entering remission had serum alkaline phosphatase levels greater than two fold the reference value, In conclusion, variants of autoimmune liver disease are common. Corticosteroid therapy can be effective in patients with features of autoimmune hepatitis and primary biliary cirrhosis, A serum alkaline phosphatase level of less than twofold the reference value characterizes corticosteroid-responsive individuals.