BACKGROUND There is limited evidence linking achievement of biochemical response with outcomes in Autoimmune Hepatitis (AIH), and it is unclear whether normalization of serum immunoglobulin G (IgG) levels influences prognosis. AIMS We aimed to investigate factors associated with death or liver transplantation in patients affected by AIH. METHODS We undertook a retrospective analysis of all AIH patients attending a tertiary liver unit since 1980. Patients not meeting established diagnostic criteria for AIH or with a follow-up shorter than 18 months were excluded. RESULTS 107 patients meeting inclusion criteria were included in the study. Mean age at diagnosis was 44 years, 29 patients (27.1%) had cirrhosis at baseline. Median follow-up was 79 months, and 70 patients (79.5%) reached biochemical response. Biochemical response was associated with reduced hazard of liver transplant or death (HR 0.07, 95% CI 0.01-0.46), whereas cirrhosis at diagnosis was an independent predictor of liver transplantation or death (Hazard ratio (HR) 11.8, 95%, confidence interval (CI) 1.18-117.4). Lack of normalization of serum IgG levels was associated with reduced 5-year transplant-free survival (95% in patients normalizing, compared to 86%, p = 0.02). CONCLUSION Normalization of serum IgG levels alone translates in better transplant-free survival in patients with AIH and should be a treatment target along with transaminases.

中文翻译：

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血清免疫球蛋白 G 水平的正常化与自身免疫性肝炎患者的无移植生存期改善有关

背景 将生化反应的实现与自身免疫性肝炎 (AIH) 的结果联系起来的证据有限，尚不清楚血清免疫球蛋白 G (IgG) 水平的正常化是否会影响预后。目的 我们旨在调查与 AIH 患者死亡或肝移植相关的因素。方法 我们对自 1980 年以来就诊于三级肝脏单位的所有 AIH 患者进行了回顾性分析。不符合 AIH 既定诊断标准或随访时间短于 18 个月的患者被排除在外。结果 107 名符合纳入标准的患者被纳入研究。诊断时的平均年龄为 44 岁，29 名患者 (27.1%) 在基线时患有肝硬化。中位随访时间为 79 个月，70 名患者（79.5%）达到生化反应。生化反应与肝移植或死亡风险降低相关（HR 0.07，95% CI 0.01-0.46），而诊断时的肝硬化是肝移植或死亡的独立预测因子（风险比 (HR) 11.8, 95%，置信区间） (CI) 1.18-117.4)。血清 IgG 水平缺乏正常化与 5 年无移植生存率降低有关（正常化患者为 95%，相比之下为 86%，p = 0.02）。结论 单独血清 IgG 水平的正常化转化为 AIH 患者更好的无移植生存率，应该与转氨酶一起作为治疗目标。血清 IgG 水平缺乏正常化与 5 年无移植生存率降低有关（正常化患者为 95%，相比之下为 86%，p = 0.02）。结论 单独血清 IgG 水平的正常化转化为 AIH 患者更好的无移植生存率，应该与转氨酶一起作为治疗目标。血清 IgG 水平缺乏正常化与 5 年无移植生存率降低有关（正常化患者为 95%，相比之下为 86%，p = 0.02）。结论 单独血清 IgG 水平的正常化转化为 AIH 患者更好的无移植生存率，应该与转氨酶一起作为治疗目标。