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Abstract：
BACKGROUND: Autoimmune liver diseases (AILD) are increasing and common forms of chronic liver disease (CLD) with different clinical responses and characteristics which can result in cirrhosis. This study aimed to investigate the natural history and characteristics of AILD in an Iranian population.
METHODS: Patients with AILD [Autoimmune Hepatitis (AIH), Primary Biliary Cholangitis (PBC), Primary Sclerosing Cholangitis (PSC) and Overlap Syndrome (OS)] referred to Middle East Liver Diseases (MELD) center, Tehran, Iran, between January 2002 and December 2022 were included in this retrospective cohort study. The main features of natural history (the trends of liver functional tests (LFT), Auto-Antibodies, response to treatment and cirrhotic status) along with demographic data were studied.
RESULTS: Two hundred sixty-five patients (160 (60.4%) AIH, 37 (14.0%) PBC, 20 (7.5%) PSC, 48 (18.1%) overlap syndrome) with a median follow-up time of 5 years (IQR 4 to 8 years) were included. Baseline laboratory tests revealed that patients with AIH exhibit elevated transaminase levels. However, patients suffering from PBC and PSC displayed increased alkaline phosphatase levels. Conversely, in overlap syndrome patients, both transaminases and alkaline phosphatase were observed at high levels. Autoantibodies represented themselves as important diagnostic markers for the AIH and PBC but not for PSC. The complete response occurred in 112 (70%) of and 28 (58.4%) patients with AIH and overlap syndrome respectively and 21 patients 11 (6.9%) of AIH and 10 (20.8%) of overlap syndrome) were non-responders. Other patients in these two categories were considered as insufficient responders. On the other side, 32 (91.9%) and 8 (40%) of patients with PBC and PSC biochemically responded to Ursodeoxycholic Acid (UDCA). Unpredictably, cirrhosis regression was observed in some AIH and PBC patients.
CONCLUSIONS: Appropriate medication management for AILD patients may leads to regression from cirrhosis and improvement of manifestations; while discontinuation of medication may cause relapses. However, patient suffering from PSC showed limited response to treatment.
METHODS: Patients with AILD [Autoimmune Hepatitis (AIH), Primary Biliary Cholangitis (PBC), Primary Sclerosing Cholangitis (PSC) and Overlap Syndrome (OS)] referred to Middle East Liver Diseases (MELD) center, Tehran, Iran, between January 2002 and December 2022 were included in this retrospective cohort study. The main features of natural history (the trends of liver functional tests (LFT), Auto-Antibodies, response to treatment and cirrhotic status) along with demographic data were studied.
RESULTS: Two hundred sixty-five patients (160 (60.4%) AIH, 37 (14.0%) PBC, 20 (7.5%) PSC, 48 (18.1%) overlap syndrome) with a median follow-up time of 5 years (IQR 4 to 8 years) were included. Baseline laboratory tests revealed that patients with AIH exhibit elevated transaminase levels. However, patients suffering from PBC and PSC displayed increased alkaline phosphatase levels. Conversely, in overlap syndrome patients, both transaminases and alkaline phosphatase were observed at high levels. Autoantibodies represented themselves as important diagnostic markers for the AIH and PBC but not for PSC. The complete response occurred in 112 (70%) of and 28 (58.4%) patients with AIH and overlap syndrome respectively and 21 patients 11 (6.9%) of AIH and 10 (20.8%) of overlap syndrome) were non-responders. Other patients in these two categories were considered as insufficient responders. On the other side, 32 (91.9%) and 8 (40%) of patients with PBC and PSC biochemically responded to Ursodeoxycholic Acid (UDCA). Unpredictably, cirrhosis regression was observed in some AIH and PBC patients.
CONCLUSIONS: Appropriate medication management for AILD patients may leads to regression from cirrhosis and improvement of manifestations; while discontinuation of medication may cause relapses. However, patient suffering from PSC showed limited response to treatment.
摘要：
背景：自身免疫性肝病（AILD）是慢性肝病（CLD）的常见形式，具有不同的临床反应和特征，可导致肝硬化。这项研究旨在调查伊朗人口中AILD的自然历史和特征。
方法：AILD患者[自身免疫性肝炎（AIH），原发性胆道胆管炎(PBC),原发性硬化性胆管炎（PSC）和重叠综合征（OS）]转诊至中东肝病（MELD）中心，德黑兰,伊朗,本回顾性队列研究纳入了2002年1月至2022年12月的队列研究.自然史的主要特征（肝功能检查（LFT）的趋势，自动抗体,对治疗和肝硬化状况的反应)以及人口统计学数据进行了研究。
结果：二百六十五名患者（160（60.4%）AIH，37（14.0%）PBC，20（7.5%）PSC，纳入48例（18.1%）重叠综合征），中位随访时间为5年（IQR4至8年）。基线实验室检查显示，AIH患者转氨酶水平升高。然而,PBC和PSC患者碱性磷酸酶水平升高.相反，在重叠综合征患者中，转氨酶和碱性磷酸酶均处于高水平.自身抗体本身是AIH和PBC的重要诊断标记，但不是PSC的重要诊断标记。AIH和重叠综合征的患者中分别有112例（70％）和28例（58.4％）完全缓解，而AIH的11例（6.9％）和重叠综合征的10例（20.8％）患者为无反应者。这两个类别中的其他患者被认为是反应不足的患者。在另一边,32（91.9％）和8（40％）的PBC和PSC患者对熊去氧胆酸（UDCA）有生化反应。不可预测的,在一些AIH和PBC患者中观察到肝硬化消退。
结论：AILD患者适当的药物管理可能导致肝硬化消退和症状改善；而停药可能导致复发。然而,PSC患者对治疗的反应有限.
方法：AILD患者[自身免疫性肝炎（AIH），原发性胆道胆管炎(PBC),原发性硬化性胆管炎（PSC）和重叠综合征（OS）]转诊至中东肝病（MELD）中心，德黑兰,伊朗,本回顾性队列研究纳入了2002年1月至2022年12月的队列研究.自然史的主要特征（肝功能检查（LFT）的趋势，自动抗体,对治疗和肝硬化状况的反应)以及人口统计学数据进行了研究。
结果：二百六十五名患者（160（60.4%）AIH，37（14.0%）PBC，20（7.5%）PSC，纳入48例（18.1%）重叠综合征），中位随访时间为5年（IQR4至8年）。基线实验室检查显示，AIH患者转氨酶水平升高。然而,PBC和PSC患者碱性磷酸酶水平升高.相反，在重叠综合征患者中，转氨酶和碱性磷酸酶均处于高水平.自身抗体本身是AIH和PBC的重要诊断标记，但不是PSC的重要诊断标记。AIH和重叠综合征的患者中分别有112例（70％）和28例（58.4％）完全缓解，而AIH的11例（6.9％）和重叠综合征的10例（20.8％）患者为无反应者。这两个类别中的其他患者被认为是反应不足的患者。在另一边,32（91.9％）和8（40％）的PBC和PSC患者对熊去氧胆酸（UDCA）有生化反应。不可预测的,在一些AIH和PBC患者中观察到肝硬化消退。
结论：AILD患者适当的药物管理可能导致肝硬化消退和症状改善；而停药可能导致复发。然而,PSC患者对治疗的反应有限.
