Nephrology and Dialysis Unit, Meyer Children's University Hospital - IRCCS, Firenze, Italy.
Department of Experimental and Clinical Medicine, University of Fireze, Firenze, Italy.
Expert Rev Clin Immunol. 2023 May;19(5):537-547. doi: 10.1080/1744666X.2023.2195627. Epub 2023 Apr 6.
IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory disease affecting multiple organs. In recent years basic and translational research has unveiled the role of different cellular subtypes and cytokines in inducing and perpetuating the pathological process, eventually leading to fibrosis of affected tissues. Hopefully, the growing knowledge of the disease pathogenesis will lead to patient-tailored treatments in the near future.
This review focuses on the most recent discoveries concerning the pathogenic mechanisms underlying IgG4-RD and highlights their potential role as specific therapeutic targets.
IgG4-RD is a systemic and multifaceted disease. Its sensitivity to glucocorticoids is well known, however new targeted therapies are emerging that can reduce glucocorticoid exposure and achieve sustained clinical responses. Clinicians managing patients with such a rare and heterogeneous disease must therefore be aware of its varied phenotype and traditional and novel therapeutic strategies.
IgG4 相关疾病（IgG4-RD）是一种罕见的影响多种器官的纤维炎症性疾病。近年来，基础和转化研究揭示了不同细胞亚型和细胞因子在诱导和持续病理过程中的作用，最终导致受影响组织的纤维化。希望对疾病发病机制的日益了解将在不久的将来导致针对患者的治疗方法。
本文综述了 IgG4-RD 发病机制的最新发现，并强调了它们作为特定治疗靶点的潜在作用。
IgG4-RD 是一种全身性和多方面的疾病。人们熟知其对糖皮质激素的敏感性，但新的靶向治疗方法正在出现，可以减少糖皮质激素的暴露并实现持续的临床反应。因此，管理此类罕见且异质性疾病的临床医生必须了解其不同的表型以及传统和新型治疗策略。
