Abe Kazumichi, Takahashi Atsushi, Nozawa Yoshihiro, Imaizumi Hiromichi, Hayashi Manabu, Okai Ken, Kanno Yukiko, Watanabe Hiroshi, Ohira Hiromasa
Department of Gastroenterology and Rheumatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, 960-1295, Japan,
Med Mol Morphol. 2014 Sep;47(3):162-8. doi: 10.1007/s00795-014-0082-z. Epub 2014 Jun 27.
Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) present with distinct clinical features. The term "PBC-AIH overlap syndrome (OS)" has been adopted to describe the condition characterized by occurrence of both PBC and AIH, although this clinical entity is difficult to define. This study aimed to assess the utility of IgG, IgM, and CD138 immunohistochemistry in the evaluation of AIH, PBC, and OS. Immunohistochemistry was performed with anti-human IgG, IgM, and CD138 to detect specific plasma cells in the liver. Predominant IgG staining was observed in AIH (85.7 %), while equivocal (46.1 %) or predominant (38.5 %) IgM staining was observed in PBC. In OS, equivocal (20 %) or predominant (80 %) IgG staining was observed. The IgM/IgG ratio was significantly higher in PBC than in AIH or OS (P < 0.005). Histological findings revealed significantly higher IgM expression in PBC at cholangitis activity grades 2-3 compared to those at cholangitis activity grades 0-1. In contrast, a significantly higher IgG expression was observed in PBC at hepatitis activity and fibrosis grades 2-3 compared to those at hepatitis activity and fibrosis grades 0-1. Taken together, periportal plasmacytic infiltrates with variable immunohistochemistry patterns of IgG and IgM expression characterized different autoimmune liver diseases.
原发性胆汁性肝硬化（PBC）和自身免疫性肝炎（AIH）具有不同的临床特征。“PBC-AIH重叠综合征（OS）”这一术语已被用于描述以同时出现PBC和AIH为特征的疾病状态，尽管这一临床实体难以明确界定。本研究旨在评估IgG、IgM和CD138免疫组化在AIH、PBC和OS评估中的作用。采用抗人IgG、IgM和CD138进行免疫组化，以检测肝脏中的特异性浆细胞。在AIH中观察到主要为IgG染色（85.7%），而在PBC中观察到不明确（46.1%）或主要为（38.5%）IgM染色。在OS中，观察到不明确（20%）或主要为（80%）IgG染色。PBC中的IgM/IgG比值显著高于AIH或OS（P<0.005）。组织学结果显示，与胆管炎活动度0-1级相比，PBC在胆管炎活动度2-3级时IgM表达显著更高。相反，与肝炎活动度和纤维化0-1级相比，PBC在肝炎活动度和纤维化2-3级时观察到显著更高的IgG表达。综上所述，具有不同IgG和IgM表达免疫组化模式的汇管区浆细胞浸润是不同自身免疫性肝病的特征。
