Department of Gastroenterology and Hepatology, Leiden University Medical Center, Leiden, 2300 RC, the Netherlands.
Department of Pathology, Leiden University Medical Center, Leiden, 2300 RC, the Netherlands.
Liver Transpl. 2020 Dec;26(12):1573-1581. doi: 10.1002/lt.25906. Epub 2020 Oct 27.
Presentation of autoimmune hepatitis (AIH) can differ from nonacute to acute autoimmune hepatitis (A-AIH) with jaundice and acute severe autoimmune hepatitis (AS-AIH) with jaundice and coagulopathy. The aim of the study was to evaluate the short-term prognosis of different presentations of AIH and the influence of liver function improvement on short-term prognosis. In this single-center retrospective cohort study, AIH patients with repeatedly tested liver function at diagnosis and during at least 1 year of follow-up were included. A-AIH was defined as bilirubin >45 µmol and international normalized ratio (INR) <1.5. AS-AIH was defined as bilirubin level >45 µmol/L and INR ≥1.5. Of the 81 included patients, 17 (21%) presented with A-AIH, and 14 (17%) presented with AS-AIH. After the start of immunosuppressive therapy, bilirubin, albumin, and INR normalized in 70%, 77%, and 69%, respectively, in a median of 2.6 months, 3 months, and 4 weeks, respectively, in patients with A-AIH and AS-AIH. Liver transplantation (LT)-free survival rate was 100% in nonacute AIH, 94% in A-AIH, and 57% in AS-AIH at 12 months after diagnosis. An increase of INR or bilirubin at 2 weeks was the best predictive factor for the need of LT within 12 months with a Youden's index of 0.85. A-AIH was present in 21%, and AS-AIH was present in 17% of AIH patients. In the majority of patients, bilirubin, albumin, and INR normalized in the first months of treatment. Deterioration of liver function after 2 weeks of treatment should lead to rapid evaluation for LT and consideration of second-line medication.
自身免疫性肝炎 (AIH) 的表现形式可以从非急性转变为伴有黄疸的急性自身免疫性肝炎 (A-AIH),或从非急性转变为伴有黄疸和凝血障碍的急性严重自身免疫性肝炎 (AS-AIH)。本研究旨在评估不同表现形式的 AIH 的短期预后,以及肝功能改善对短期预后的影响。在这项单中心回顾性队列研究中,纳入了在诊断时和至少 1 年随访期间反复检测肝功能的 AIH 患者。A-AIH 的定义为胆红素>45µmol/L 和国际标准化比值 (INR)<1.5。AS-AIH 的定义为胆红素水平>45µmol/L 和 INR≥1.5。在纳入的 81 例患者中,17 例(21%)表现为 A-AIH,14 例(17%)表现为 AS-AIH。在开始免疫抑制治疗后,A-AIH 和 AS-AIH 患者的胆红素、白蛋白和 INR 分别在中位数为 2.6 个月、3 个月和 4 周时分别恢复正常,恢复率分别为 70%、77%和 69%。在非急性 AIH、A-AIH 和 AS-AIH 中,诊断后 12 个月时无肝移植(LT)生存率分别为 100%、94%和 57%。2 周时 INR 或胆红素升高是 12 个月内需要 LT 的最佳预测因素,其 Youden 指数为 0.85。21%的 AIH 患者表现为 A-AIH,17%的 AIH 患者表现为 AS-AIH。在大多数患者中,胆红素、白蛋白和 INR 在治疗的第一个月内恢复正常。治疗 2 周后肝功能恶化应导致迅速评估 LT,并考虑二线药物。