Floreani Annarosa, Okazaki Kazuichi, Uchida Kazushige, Gershwin M Eric
Scientific Consultant IRCCS Negrar, Verona, Italy.
Senior Scholar, University of Padova, Italy.
J Transl Autoimmun. 2020 Dec 19;4:100074. doi: 10.1016/j.jtauto.2020.100074. eCollection 2021.
IgG4-related disease (IgG4-RD) represents an immune-mediated fibroinflammatory condition with peculiar histopathologic changes that can affect various organs. In 2012 its unified nomenclature was published, which allows to abandon other synonymous names. Up to now, only little is known about its epidemiology around the world. However, although it is generally considered a rare condition, the number of patients with IgG4-RD is increasing enormously. Likewise, the annual number of publications on this subject has increased progressively. The spectrum of clinical manifestations in IgG4-RD is highly variable, depending on the severity of the disease as well as the presence of organ(s) involvement. This review gives an overview on changing epidemiology of IgG4-RD focusing the attention on the large cohorts of patients published in the literature.
IgG4相关性疾病（IgG4-RD）是一种免疫介导的纤维炎症性疾病，具有独特的组织病理学变化，可累及多个器官。2012年发布了其统一命名法，摒弃了其他同义名称。到目前为止，全球范围内对其流行病学了解甚少。然而，尽管通常认为这是一种罕见疾病，但IgG4-RD患者的数量正在大幅增加。同样，关于该主题的年度出版物数量也在逐步增加。IgG4-RD的临床表现谱高度可变，取决于疾病的严重程度以及器官受累情况。本综述概述了IgG4-RD不断变化的流行病学，重点关注文献中发表的大量患者队列。
