Department of Medicine and Research Laboratory of Internal Medicine, National Expertise Center of Greece in Autoimmune Liver Diseases, General University Hospital of Larissa, Larissa, Greece; European Reference Network on Hepatological Diseases (ERN RARE-LIVER), General University Hospital of Larissa, Larissa, Greece.
Department of Medicine and Research Laboratory of Internal Medicine, National Expertise Center of Greece in Autoimmune Liver Diseases, General University Hospital of Larissa, Larissa, Greece
Pol Arch Intern Med. 2022 Sep 26;132(9). doi: 10.20452/pamw.16334. Epub 2022 Sep 7.
Autoimmune hepatitis (AIH) is an acute or chronic inflammatory disease of the liver caused by an immune response of unknown origin. It affects people from all ethnic groups irrespective of age or sex. AIH is characterized by hyperglobulinemia, presence of circulating autoantibodies, and liver inflammation. The clinical picture of the disease varies from asymptomatic or mild to severe acute hepatitis or liver failure. A timely and prompt diagnosis is of utmost importance to prevent progression to advanced liver disease by immediate initiation of immunosuppressive treatment. So far, several diagnostic scoring systems have been proposed, which incorporated demographic data as well as biochemical, clinical, and histological characteristics of the disease. However, due to the high heterogeneity of the disease presentation, diagnosis of AIH remains challenging. Most patients initially respond to first‑ line treatment, which consists of corticosteroids combined with azathioprine or mycophenolate mofetil. However, insufficient response to the treatment and intolerance due to side effects are common, so a significant proportion of patients require second- and / or third‑ line therapies. Herein, we review the challenges and recent advances in AIH diagnosis and management.
自身免疫性肝炎（AIH）是一种由未知来源的免疫反应引起的肝脏急性或慢性炎症性疾病。它影响所有种族的人群，无论年龄或性别。AIH 的特征是高球蛋白血症、循环自身抗体的存在和肝脏炎症。该疾病的临床表现从无症状或轻度到严重的急性肝炎或肝功能衰竭不等。及时和迅速的诊断对于通过立即开始免疫抑制治疗来防止进展为晚期肝病至关重要。迄今为止，已经提出了几种诊断评分系统，这些系统纳入了人口统计学数据以及疾病的生化、临床和组织学特征。然而，由于疾病表现的高度异质性，AIH 的诊断仍然具有挑战性。大多数患者最初对一线治疗有反应，一线治疗包括皮质类固醇联合硫唑嘌呤或霉酚酸酯。然而，由于副作用导致治疗反应不足和不耐受很常见，因此很大一部分患者需要二线和/或三线治疗。在此，我们回顾 AIH 诊断和治疗方面的挑战和最新进展。
