IgG4相关性疾病中医病因病机、辨证分型与中医药干预研究进展

Research Progress on Etiology, Pathogenesis, Syndrome Differentiation and Traditional Chinese Medicine Intervention of IgG4-Related Disease

摘要: IgG4相关性疾病(IgG4-related disease, IgG4-RD)是一种以血清IgG4水平异常升高、多器官IgG4阳性浆细胞浸润及席纹状纤维化为病理特征的慢性自身免疫性疾病，胰腺、胆管、泪腺、唾液腺、肾脏及腹膜后等部位均可受累。现代医学以糖皮质激素作为一线用药，但停药后复发率偏高，长期应用所致不良反应亦不容忽视。近年来，中医药对本病的辨治积累了较为丰富的经验。本文拟从中医病名归属、病因病机认识、辨证分型规律、单系统亚型辨治要点、中西医结合优势及作用机制等方面，对近十余年来相关文献进行系统梳理与归纳，以期为临床诊疗及后续研究提供参考。

Abstract: IgG4-related disease (IgG4-RD) is a chronic autoimmune disorder pathologically characterized by elevated serum IgG4 levels, infiltration of IgG4-positive plasma cells in multiple organs, and storiform fibrosis, which may involve the pancreas, bile ducts, lacrimal glands, salivary glands, kidneys, retroperitoneum, and other sites. Glucocorticoids remain the first-line therapy in modern medicine, yet high relapse rates after withdrawal and notable adverse effects of long-term use remain clinical challenges. In recent years, traditional Chinese medicine (TCM) has accumulated considerable experience in the differentiation and treatment of this disease. This article systematically reviews relevant literature over the past decade from the perspectives of TCM disease name attribution, etiology and pathogenesis, syndrome differentiation patterns, single-organ subtype management, advantages of integrated Chinese-Western therapy, and potential mechanisms, aiming to provide references for clinical practice and future research.