IgG4 相关性疾病的诊疗研究现状

Recent progress in the diagnosis and management of IgG4-related diseases

摘要

IgG4 相关性疾病 (IgG4-RD) 是一种全身性自身免疫性疾病, 主要特征是慢性炎症和纤维化改变, IgG4-RD 的病因和发病机制尚未完全阐明, 可能与炎症因子、细胞免疫及肠道菌群失调等有关, 需要结合血清学改变、影像学表现和症状等综合分析后诊断, 该疾病治疗手段主要包括糖皮质激素治疗、免疫抑制剂、抗体靶向治疗、调节肠道菌群以及药物联合治疗等。本文旨在阐述 IgG4-RD 的发病机制、临床症状、诊疗方案的研究进展, 为 IgG4-RD 的临床诊疗提供新思路。

Abstract

IgG4-related disease (IgG4-RD) is a systemic autoimmune disorder condition largely distinguished by persistent inflammation and fibrotic alterations. The etiology and pathogenesis of IgG4-RD are not fully elucidated but may involve inflammatory mediators, cellular immunity, and dysbiosis of the gut microbiota. Diagnosis necessitates a thorough evaluation of serological alterations, imaging results, and clinical manifestations. This disease can impact several organs and tissues, with clinical presentations classified into systemic symptoms, organ-specific symptoms, and mass-like lesions. Treatment modalities encompass glucocorticoids, immunosuppressants, antibody-targeted treatments, modification of gut microbiota, and prospective combination medicines. This article is to examine recent developments in the pathophysiology, clinical symptoms, diagnosis, and treatment approaches of IgG4-RD, offering fresh perspectives for future clinical management.

关键词

IgG4 相关性疾病 / 发病机制 / 诊疗进展 / 研究现状

Key words

IgG4-related diseases / Pathogenesis / Advances in diagnosis and treatment / Current state of research

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