The Geoepidemiology and Clinical Aspects of IgG4-Related Disease.
The Geoepidemiology and Clinical Aspects of IgG4-Related Disease.
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IgG4 相关疾病的地理流行病学和临床方面。
DOI：
10.1055/s-0036-1584323
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发表时间：
2016
期刊：
影响因子：
--
通讯作者：
Okazaki K
中科院分区：
文献类型：
--
作者：
Uchida K;Tanaka T;Gershwin ME;Okazaki K
免疫球蛋白G4相关疾病(IgG4-RD)是一种新近报道的全身性炎症性疾病，以血清IgG4浓度升高、淋巴浆细胞浸润性病变、斑片状纤维化和闭塞性静脉炎为特征。然而，尽管IgG4-RD已得到越来越多的认识，但IgG4-RD患者的数量仍不清楚。多项研究的数据表明，T辅助细胞2型(Th2-)占优势的免疫反应导致Th2细胞因子的过度产生，然后进展为IgG4-RD。糖皮质激素是治疗IgG4-RD最常见的治疗方法，总体来说，患者的反应良好--这是IgG4-RD的一个特征。然而，在逐渐减少糖皮质激素治疗期间复发是很常见的。糖皮质激素后的二线治疗包括免疫抑制剂。虽然长期结果仍不清楚，但人们对免疫球蛋白-RD和恶性肿瘤之间的关系越来越感兴趣。在这篇综述中，作者对IgG4-RD的地质流行病学、发病机制、诊断特征、治疗和预后进行了详细的概述。
Immunoglobulin G4-related disease (IgG4-RD) is a recently described systemic inflammatory disease characterized by increased serum IgG4 concentrations, lymphoplasmacytic infiltrations, storiform fibrosis, and obliterative phlebitis. However, although IgG4-RD has become increasingly recognized, the number of patients with IgG4-RD remains unclear. Data from several studies indicate that patients who have a T-helper type 2 (Th2-) dominant immune response, which leads to the hyperproduction of Th2 cytokines, then progress to IgG4-RD. Glucocorticoids are the most common treatment for IgG4-RD and generally, patients have a good response—a characteristic of IgG4-RD. However, relapses during the tapering of glucocorticoid therapy are common. Second-line therapy after glucocorticoids includes immunosuppressant agents. Although the long-term outcome still remains unclear, there is increased interest in the relationships between IgG-RD and malignancies. In this review, the authors provide a detailed overview of the geoepidemiology, pathogenesis, diagnostic features, treatment, and prognosis of IgG4-RD.