Hatoum Sara, Rockey Don C
Digestive Disease Research Center, Medical University of South Carolina, Charleston, South Carolina, USA.
Eur J Gastroenterol Hepatol. 2024 Jun 1;36(6):742-749. doi: 10.1097/MEG.0000000000002714. Epub 2024 Mar 28.
Autoimmune hepatitis is an immune-mediated liver disease that results in hepatic inflammation and subsequent fibrosis. We aimed to assess the natural history of autoimmune hepatitis in patients who had cirrhosis at the time of diagnosis.
We examined consecutive patients with autoimmune hepatitis (based on the revised International Autoimmune Hepatitis Group criteria) and cirrhosis who had long-term follow-up between 2012 and 2018. Complete clinical data, including longitudinal data, was obtained for each patient to determine clinical and biochemical outcomes. Decompensating events were defined as complications of portal hypertension.
Thirty-four patients presenting with autoimmune hepatitis induced cirrhosis (age 50, 17-81; 71% women) were followed for an average of 8 years post-diagnosis. Fourteen (41%) patients had a decompensating event at diagnosis. All patients were begun on treatment; index decompensating events resolved in all patients. Twenty-six (76%) patients had normalization of transaminases; in this group, 4 (15%) patients developed one or more new decompensating events and 1 patient (4%) died. Of the 8 (24%) patients who did not have transaminase normalization, 6 (75%) developed one or more new decompensating events and 5 (62%) died or underwent liver transplant. There was a significant association between achieving normalization of transaminases and protection from developing a decompensating event ( P = 0.003) and liver transplant or death ( P = 0.001).
Most patients with autoimmune hepatitis with cirrhosis at presentation achieved normalization of transaminases with treatment and rarely developed further decompensating events. We speculate that some of these patients had stabilization or reversal of portal hypertension.
自身免疫性肝炎是一种免疫介导的肝脏疾病，可导致肝脏炎症及随后的纤维化。我们旨在评估诊断时已出现肝硬化的自身免疫性肝炎患者的自然病史。
我们对2012年至2018年期间接受长期随访的自身免疫性肝炎（根据修订后的国际自身免疫性肝炎小组标准）合并肝硬化的连续患者进行了检查。获取了每位患者的完整临床数据，包括纵向数据，以确定临床和生化结果。失代偿事件定义为门静脉高压并发症。
34例出现自身免疫性肝炎所致肝硬化的患者（年龄50岁，17 - 81岁；71%为女性）在诊断后平均随访8年。14例（41%）患者在诊断时发生失代偿事件。所有患者均开始接受治疗；所有患者的首次失代偿事件均得到缓解。26例（76%）患者转氨酶恢复正常；在该组中，4例（15%）患者发生了一次或多次新的失代偿事件，1例（4%）患者死亡。在8例（24%）转氨酶未恢复正常的患者中，6例（75%）发生了一次或多次新的失代偿事件，5例（62%）死亡或接受了肝移植。转氨酶恢复正常与预防失代偿事件（P = 0.003）以及肝移植或死亡（P = 0.001）之间存在显著关联。
大多数初诊时患有自身免疫性肝炎合并肝硬化的患者经治疗后转氨酶恢复正常，很少发生进一步的失代偿事件。我们推测这些患者中的一些人门静脉高压得到了稳定或逆转。
