Chaudhuri Urmimala, Forrest Jonathan R, Sastry Karthik, Reagans Ryan, Agrawal Sangeeta
Internal Medicine Residency Program, Wright State University, Dayton, USA.
Boonshoft School of Medicine, Wright State University, Dayton, USA.
Cureus. 2025 May 19;17(5):e84405. doi: 10.7759/cureus.84405. eCollection 2025 May.
The coexistence of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) is termed AIH-PBC overlap syndrome, a rare but recognized clinical entity. Clinical presentation is often non-specific, including fatigue, myalgias, arthralgias, and cholestatic liver function test (LFT) abnormalities. Diagnosis is based on biochemical, histologic, and immunologic features commonly using the well-established Paris criteria. While the exact etiology is unclear, immune dysregulation triggered by medications may play a role. We present the case of a 51-year-old male patient with hypertension and type 2 diabetes mellitus who developed elevated LFTs two weeks after starting hydralazine. Serologies revealed positive antimitochondrial antibody (AMA), antinuclear antibody (ANA), and anti-smooth muscle antibody (ASMA) while viral and acetaminophen toxicity were ruled out. An initial liver biopsy demonstrated mixed portal and lobular inflammation without definitive features of AIH or PBC. Despite discontinuing hydralazine, LFTs remained elevated. A repeat liver biopsy revealed florid duct lesions and interface hepatitis. Based on the Paris criteria and clinical judgement, the patient was diagnosed with AIH-PBC overlap syndrome. Treatment with prednisone and ursodiol led to near normalization of LFTs. While DILI-induced AIH-PBC overlap has previously been reported with agents like infliximab and tuberculosis therapies, this is the first reported case potentially triggered by hydralazine. Immune dysregulation may have resulted from hepatic injury induced by hydralazine. This case highlights the importance of considering drug-induced liver injury as a potential precipitant of AIH-PBC overlap and the need for early recognition and treatment.
自身免疫性肝炎（AIH）与原发性胆汁性胆管炎（PBC）并存被称为AIH-PBC重叠综合征，这是一种罕见但已被认可的临床实体。临床表现通常不具有特异性，包括疲劳、肌痛、关节痛和胆汁淤积性肝功能检查（LFT）异常。诊断基于生化、组织学和免疫学特征，通常采用已确立的巴黎标准。虽然确切病因尚不清楚，但药物引发的免疫失调可能起了作用。我们报告一例51岁男性患者，患有高血压和2型糖尿病，在开始使用肼苯哒嗪两周后出现肝功能检查指标升高。血清学检查显示抗线粒体抗体（AMA）、抗核抗体（ANA）和抗平滑肌抗体（ASMA）呈阳性，同时排除了病毒感染和对乙酰氨基酚毒性。初次肝活检显示门静脉和小叶混合性炎症，无AIH或PBC的确切特征。尽管停用了肼苯哒嗪，但肝功能检查指标仍居高不下。再次肝活检显示明显的胆管病变和界面性肝炎。根据巴黎标准和临床判断，该患者被诊断为AIH-PBC重叠综合征。使用泼尼松和熊去氧胆酸治疗使肝功能检查指标接近正常。虽然之前已有使用英夫利昔单抗和结核病治疗药物等导致药物性肝损伤（DILI）引发AIH-PBC重叠的报道，但这是首例可能由肼苯哒嗪引发的病例。免疫失调可能是由肼苯哒嗪引起的肝损伤导致的。该病例凸显了将药物性肝损伤视为AIH-PBC重叠潜在诱因的重要性，以及早期识别和治疗的必要性。
