A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details.

A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details.
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一种新的临床实体,IgG4 相关疾病 (IgG4RD):一般概念和细节。

DOI:
10.1007/s10165-011-0508-6
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发表时间:
2012-02
影响因子:
2.2
通讯作者:
Inoue, Dai
Inoue, Dai
中科院分区:
医学3区
文献类型:
--
作者:
Umehara, Hisanori;Okazaki, Kazuichi;Masaki, Yasufumi;Kawano, Mitsuhiro;Yamamoto, Motohisa;Saeki, Takako;Matsui, Shoko;Sumida, Takayuki;Mimori, Tsuneyo;Tanaka, Yoshiya;Tsubota, Kazuo;Yoshino, Tadashi;Kawa, Shigeyuki;Suzuki, Ritsuro;Takegami, Tsutomu;Tomosugi, Naohisa;Kurose, Nozomu;Ishigaki, Yasuhito;Azumi, Atsushi;Kojima, Masaru;Nakamura, Shigeo;Inoue, Dai

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IgG4相关疾病(IgG4RD)是一种以血清IgG4浓度升高和IgG4阳性浆细胞肿胀或组织浸润为特征的新型临床疾病。IgG4RD可存在于一定比例的患有多种疾病的患者中,包括Mikulicz病、自身免疫性胰腺炎、垂体炎、Riedel甲状腺炎、间质性肺炎、间质性肾炎、前列腺炎、淋巴结病、腹膜后纤维化、炎性主动脉瘤和炎性假瘤。尽管IgG4RD形成了一个独特的、临床上独立的疾病类别,并作为一个新的临床实体引起了人们的强烈关注,但仍有许多问题和问题有待阐明,包括其发病机制、诊断标准的建立以及IgG4的作用。在这里,我们描述的概念IgG4RD和最新的信息,这种新出现的疾病实体。
IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz’s disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm, and inflammatory pseudotumor. Although IgG4RD forms a distinct, clinically independent disease category and is attracting strong attention as a new clinical entity, many questions and problems still remain to be elucidated, including its pathogenesis, the establishment of diagnostic criteria, and the role of IgG4. Here we describe the concept of IgG4RD and up-to-date information on this emerging disease entity.
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