Abstract：
A prompt response to glucocorticoids is a clinical hallmark of IgG4-related disease. However, manifestations characterised by prominent tissue fibrosis on histological examination can be less responsive to glucocorticoid therapy than other types of IgG4-related disease. These manifestations include retroperitoneal fibrosis, fibrosing mediastinitis, Riedel thyroiditis, orbital pseudotumor, and hypertrophic pachymeningitis, among others. To explain this discrepancy, a preliminary distinction into proliferative and fibrotic phenotypes of IgG4-related disease has been proposed on the basis of clinical presentation, pathological features, and response to immunosuppressive therapy. Implications of this classification for patient management remain an important area of investigation. In this Series paper, we aim to dissect the pathophysiology of tissue fibrosis in IgG4-related disease and discuss how clinicians should approach the management of fibrotic manifestations of IgG4-related disease based on the most recent diagnostic and therapeutic developments.
摘要：
对糖皮质激素的迅速反应是IgG4相关疾病的临床标志。然而,与其他类型的IgG4相关疾病相比,以组织学检查中明显的组织纤维化为特征的表现对糖皮质激素治疗的反应较小.这些表现包括腹膜后纤维化,纤维化纵隔炎，里德尔甲状腺炎,轨道假瘤,肥厚性硬脑膜炎,在其他人中。为了解释这种差异，根据临床表现对IgG4相关疾病的增殖性和纤维化表型进行了初步区分,病理特征,和对免疫抑制治疗的反应。这种分类对患者管理的影响仍然是研究的重要领域。在本系列论文中，我们旨在剖析IgG4相关疾病中组织纤维化的病理生理学,并根据最新的诊断和治疗进展,讨论临床医生应如何处理IgG4相关疾病的纤维化表现.
