罗道病与IgG4相关性疾病的研究进展及相关性

Research Progress and Correlation between Rosai-Dorfman Disease and IgG4 Related Diseases

摘要: 罗道病(Rosai-Dorfman, RDD)是一种伴有大量淋巴结病的组织细胞增生症，是一种病因不明的特殊组织细胞样细胞的特发性增生。大多数RDD患者表现为多发性淋巴结病，少部分RDD也涉及多种结外部位。IgG4相关性疾病(IgG4-related disease, IgG4-RD)是一种免疫介导的疾病，与纤维炎性病变相关，可发生在几乎全身任何解剖部位，可以累及一个或多个器官，产生炎性或弥漫性增大的肿块。RDD和IgG4-RD都是罕见疾病，且两种疾病临床表现多样、没有特异性、没有明确的诊断标准，患者常因不同的临床表现而就诊于不同专科，由于单一科室诊治的片面性，造成误诊和漏诊。在某些情况下，RDD可能会在临床和病理上模仿IgG4-RD，是一种临床罕见且令人困惑的疾病。本文就RDD和IgG4-RD的临床表现、诊断、治疗、两者之间的相关性作简要综述。

Abstract: Rosai-Dorfman (RDD) is a kind of histiocytosis accompanied by a large number of lymphadenopathy, which is an idiopathic hyperplasia of special histiocyte like cells with unknown etiology. Most pa-tients with RDD show multiple lymphadenopathy, and a small number of RDD also involve multiple extranodal sites. IgG4 related disease (IgG4-RD) is an immune-mediated disease, which is related to fibroinflammatory lesions. It can occur in almost any anatomical part of the body, involving one or more organs, and producing inflammatory or diffusely enlarged masses. RDD and IgG4-RD are rare diseases, and the clinical manifestations of the two diseases are diverse, which have no specificity and clear diagnostic criteria. Patients often go to different specialties for different clinical manifes-tations. Due to the one-sided diagnosis and treatment of a single department, misdiagnosis and missed diagnosis are caused. In some cases, RDD may mimic IgG4-RD clinically and pathologically, which is a rare and puzzling disease. This article briefly reviews the clinical manifestations, diagno-sis, treatment and correlation between RDD and IgG4-RD.