Delbrune Malique, Wongjarupong Nicha, Aby Elizabeth S, Iwamoto Carlos, Hassan Mohamed
Gastroenterology, Hepatology and Nutrition, University of Minnesota, Minneapolis, USA.
Gastroenterology, Hepatology and Nutrition/ Transplant Hepatology, University of Minnesota, Minneapolis, USA.
Cureus. 2023 Aug 24;15(8):e44066. doi: 10.7759/cureus.44066. eCollection 2023 Aug.
Immunoglobulin G4-related disease (IgG4-RD) is a unique immunological disease that can impact multiple organs including a formation of a hepatic inflammatory pseudotumor (IPT). We present a case of a 67-year-old male with a history of chronic viral hepatitis C infection who had an accidental finding on magnetic resonance imaging (MRI) of a liver arterially enhancing lesion. With an extensive work-up, immunohistochemical stains for immunoglobulin G of the liver lesion was performed and showed markedly increased IgG4-positive plasma cells (> 50/HPF), which was consistent with hepatic inflammatory pseudotumor related to IgG4-RD. The patient was treated with prednisone with a complete resolution of the hepatic lesion. The diagnosis of hepatic IPT and IgG4-RD requires a high degree of clinical suspicion and coordination with a multi-disciplinary team, including pathologists. Early tissue acquisition and staining for IgG4 was essential for the early diagnosis and treatment in this case. We also provide a comprehensive summary of published reports of IgG4-RD presenting with IPT.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种独特的免疫性疾病，可累及多个器官，包括形成肝脏炎性假瘤（IPT）。我们报告一例67岁男性，有慢性丙型病毒性肝炎感染史，其在肝脏磁共振成像（MRI）检查时意外发现一个动脉期强化的肝脏病变。经过广泛检查，对肝脏病变进行了免疫球蛋白G免疫组化染色，结果显示IgG4阳性浆细胞显著增多（>50/高倍视野），这与IgG4-RD相关的肝脏炎性假瘤一致。患者接受泼尼松治疗后肝脏病变完全消退。肝脏IPT和IgG4-RD的诊断需要高度的临床怀疑，并与包括病理学家在内的多学科团队协作。在本例中，早期获取组织并进行IgG4染色对于早期诊断和治疗至关重要。我们还对已发表的IgG4-RD伴IPT的报告进行了全面总结。
