■自身免疫性肝炎(AIH)的治疗目标是诱导缓解以防止肝纤维化的发展,肝硬化,及其相关并发症。已经使用了治疗反应和缓解的各种定义。国际自身免疫性肝炎组织(IAIHG)最近定义了治疗反应的共识标准。我们旨在验证我们队列中的IAIHG反应标准,并建立与生存终点的相关性。
■我们进行了回顾性研究,比利时一个三级和七个二级保健中心的多中心队列研究。符合条件的患者在数据收集时年龄至少为18岁,并且通过简化的IAIHG评分≥6被诊断为AIH。根据IAIHG共识标准将完全生化反应(CBR)定义为在治疗的前6个月内转氨酶和血清IgG的正常化。主要终点是肝脏相关生存-定义为肝脏相关死亡或肝移植的自由。次要终点是总死亡率和无移植生存率。比较获得CBR的患者和反应不足的患者的预后。
■可以在200例AIH患者中确定生化反应状态:128例(64.0%)个体达到CBR。未达到CBR的患者在初始组织学上更频繁地出现肝硬化(22.2%vs.10.9%,p=0.036)。26例患者(13.0%)发生肝脏相关死亡率或肝移植作为主要结局。实现CBR的患者表现出上肝相关(风险比0.118;95%CI0.052-0.267;p<0.0001)和总体(风险比0.253;95%CI0.111-0.572;p=0.0003)生存率。
■我们外部验证了IAIHG关于CBR的共识标准,并确认了它们与多中心生存终点的相关性,真实世界的队列。AIH患者以CBR为中间终点,具有明显优越的肝脏相关和总生存率。
■皮质类固醇仍然是诱导自身免疫性肝炎(AIH)疾病活动缓解的治疗基石,大多数患者需要长期皮质类固醇治疗以达到持续缓解。多年来,对治疗反应的定义各不相同,并且需要持续使用中间终点以促进非皮质类固醇治疗自身免疫性肝炎的进展.国际自身免疫性肝炎组织(IAIHG)定义了AIH治疗终点的共识标准,需要进一步的外部验证。这里,我们证明了IAIHG共识标准的有用性,并证实了它们与主要终点的相关性,例如多中心的肝脏相关存活率和天然肝脏存活率,真实世界的设置。未来研究的设计可以依靠IAIHG共识标准作为中间终点。
UNASSIGNED: The goal of treatment in autoimmune hepatitis (AIH) is induction of remission to prevent the development of liver fibrosis, cirrhosis, and its related complications. Various definitions of treatment response and remission have been used. The International Autoimmune Hepatitis Group (IAIHG) recently defined consensus criteria for treatment response. We aimed to validate the IAIHG response criteria in our cohort and establish correlations with survival endpoints.
UNASSIGNED: We performed a retrospective, multicentric cohort study in one tertiary and seven secondary care centres in Belgium. Eligible patients were at least 18 years of age at data collection and were diagnosed with AIH by a simplified IAIHG score of ≥6. Complete biochemical response (CBR) was defined according to the IAIHG consensus criteria as normalisation of transaminases and serum IgG within the first 6 months of treatment. The primary endpoint was liver-related survival - defined as freedom from liver-related death or liver transplantation. Secondary endpoints were overall mortality and transplant-free survival. Outcomes were compared between patients attaining CBR and those with insufficient response.
UNASSIGNED: Biochemical response status could be determined in 200 patients with AIH: CBR was achieved in 128 (64.0%) individuals. Patients not achieving CBR more frequently presented with cirrhosis on initial histology (22.2% vs. 10.9%, p = 0.036). Liver-related mortality or liver transplantation as a primary outcome occurred in 26 patients (13.0%). Patients achieving CBR exhibited superior liver-related (hazard ratio 0.118; 95% CI 0.052-0.267; p <0.0001) and overall (hazard ratio 0.253; 95% CI 0.111-0.572; p = 0.0003) survival.
UNASSIGNED: We externally validated the IAIHG consensus criteria for CBR and confirmed their correlation with survival endpoints in a multicentric, real-world cohort. Patients with AIH achieving CBR as an intermediate endpoint have significantly superior liver-related and overall survival.
UNASSIGNED: Corticosteroids remain the cornerstone of treatment to induce remission of disease activity in autoimmune hepatitis (AIH), and the majority of patients require long-term corticosteroid treatment to achieve sustained remission. Definitions of response to treatment have varied over the years, and consistently used intermediate endpoints are needed to facilitate advancements in non-corticosteroid treatment for autoimmune hepatitis. The International Autoimmune Hepatitis Group (IAIHG) defined consensus criteria on endpoints in the treatment of AIH, for which further external validation is needed. Here, we demonstrate the usefulness of the IAIHG consensus criteria and corroborate their correlation to primary endpoints, such as liver-related survival and native liver survival in a multicentric, real-world setting. The design of future studies can rely on the IAIHG consensus criteria as intermediate endpoints.