Phan Tina C L, Blondeau-Lecomte Esther, Jackson Christopher D
Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Department of Medicine, University of Tennessee Health Science Center, Memphis, TN, USA.
J Community Hosp Intern Med Perspect. 2025 Jul 3;15(4):50-53. doi: 10.55729/2000-9666.1501. eCollection 2025.
Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease characterized by the presence of circulating autoantibodies. The spectrum of disease manifestations extends from asymptomatic cases to mild symptoms and, in rare instances, acute liver failure. AIH is a diagnosis of exclusion, supported by the detection of autoantibodies such as anti-smooth muscle antibody (ASMA). This case describes a 43-year-old female with myasthenia gravis, receiving monthly intravenous immunoglobulin (IVIG) infusions, who presented with persistently elevated liver enzymes and mildly elevated ASMA titers across multiple clinical encounters. A liver biopsy revealed severe acute hepatitis. While drug-induced liver injury (DILI) secondary to IVIG was initially considered the leading diagnosis, the persistence of elevated liver enzymes over two months despite discontinuation of IVIG made the diagnosis of AIH more likely and brought attention to the diagnostic challenges associated with AIH. Although ASMA is a hallmark serologic marker for AIH, it only has a moderate sensitivity of 59 %. Furthermore, IVIG administration may interfere with autoimmune testing, potentially leading to false-negative results. This case illustrates the complexity of interpreting autoimmune serologies and emphasizes the need for comprehensive diagnostic approach. It also highlights the importance of recognizing cognitive biases, such as premature diagnostic closure, that can hinder accurate diagnosis.
自身免疫性肝炎（AIH）是一种慢性炎症性肝病，其特征是存在循环自身抗体。疾病表现范围从无症状病例到轻度症状，在罕见情况下会发展为急性肝衰竭。AIH是一种排除性诊断，抗平滑肌抗体（ASMA）等自身抗体的检测对其有支持作用。本病例描述了一名43岁患有重症肌无力的女性，每月接受静脉注射免疫球蛋白（IVIG）治疗，在多次临床就诊中出现肝酶持续升高和ASMA滴度轻度升高。肝脏活检显示为严重急性肝炎。虽然最初认为IVIG继发的药物性肝损伤（DILI）是主要诊断，但尽管停用IVIG，肝酶仍持续升高两个月，这使得AIH的诊断更有可能，并引起了对与AIH相关的诊断挑战的关注。虽然ASMA是AIH的标志性血清学标志物，但其敏感性仅为中等水平，为59%。此外，IVIG给药可能会干扰自身免疫检测，潜在地导致假阴性结果。本病例说明了解释自身免疫血清学的复杂性，并强调了采用综合诊断方法的必要性。它还突出了认识到认知偏差（如过早诊断封闭）的重要性，这些偏差可能会阻碍准确诊断。
