Minamino Hiroto, Inaba Hidefumi, Ariyasu Hiroyuki, Furuta Hiroto, Nishi Masahiro, Yoshimasu Takashi, Nishikawa Akinori, Nakanishi Masanori, Tsuchihashi Shigeki, Kojima Fumiyoshi, Murata Shin-Ichi, Inoue Gen, Akamizu Takashi
The First Department of Medicine, Wakayama Medical University, 811-1, Kimiidera, Wakayama, 641-8509, Japan; Department of Diabetes and Endocrinology, Wakayama Red Cross Hospital, Wakayama, Japan.
The First Department of Medicine, Wakayama Medical University , 811-1, Kimiidera, Wakayama, 641-8509 , Japan.
Endocrinol Diabetes Metab Case Rep. 2016;2016:160004. doi: 10.1530/EDM-16-0004. Epub 2016 Feb 19.
A 73-year-old man with Hashimoto's thyroiditis (HT) suffered from purpura on the lower legs. He was diagnosed with IgG4-related disease (IgG4-RD) with serum IgG4 elevation and dacryo-sialadenitis confirmed histologically. Serum Th2 and Treg cytokines, interleukin 7 (IL7), IL8 and Th2 chemokine levels were elevated, while skewed Th1 balance was seen in fluorescence-activated cell sorting (FACS). Therefore, preferential Th1 balance in HT appeared to be followed by IgG4-RD characterized with Th2 and Treg polarization. The commencement of steroid therapy dramatically exacerbated clinical manifestations including IgG4-RD-associated HT. The measurement of cytokine and chemokine levels as well as FACS analysis in the development of IgG4-RD seemed to be beneficial. In conclusion, an innovative association of HT, IgG4-RD and vasculitis was observed. This report also offers novel diagnostic and therapeutic approaches for IgG4-RD.
Recently, a subtype of HT has been considered to be a thyroid manifestation of IgG4-RD, although the etiology of IgG4-RD is not established yet.Immunologically a close association between HT and vasculitis was reported.Leukocytoclastic vasculitis is a rare skin presentation of IgG4-RD.In the current case, during the course of HT, IgG4-RD and leukocytoclastic vasculitis occurred; thus, innate immunity and acquired immunity seem to be involved in the development of IgG4-RD.The measurement of cytokine and chemokines appeared to be beneficial in the development of IgG4-RD.Remarkably, effectiveness of steroid therapy for HT suggested presence of IgG4-RD-associated HT. Therefore, this report highlights the pathogenesis of IgG4-RD and proposes novel therapeutic mechanisms. Clinicians should pay attention to the development of IgG4-RD and vasculitis during long course of HT.
一名73岁患有桥本甲状腺炎（HT）的男性小腿出现紫癜。他被诊断为IgG4相关性疾病（IgG4-RD），血清IgG4升高，组织学证实有泪腺和唾液腺炎。血清Th2和调节性T细胞（Treg）细胞因子、白细胞介素7（IL7）、IL8和Th2趋化因子水平升高，而在荧光激活细胞分选（FACS）中可见Th1平衡倾斜。因此，HT中优先出现的Th1平衡似乎随后出现了以Th2和Treg极化特征的IgG4-RD。类固醇治疗的开始显著加重了包括IgG4-RD相关HT在内的临床表现。在IgG4-RD的发展过程中测量细胞因子和趋化因子水平以及进行FACS分析似乎是有益的。总之，观察到HT、IgG4-RD和血管炎之间存在一种新的关联。本报告还为IgG4-RD提供了新的诊断和治疗方法。
最近，尽管IgG4-RD的病因尚未明确，但HT的一种亚型已被认为是IgG4-RD的甲状腺表现。在免疫学上，有报道称HT与血管炎之间存在密切关联。白细胞破碎性血管炎是IgG4-RD罕见的皮肤表现。在当前病例中，在HT病程中发生了IgG4-RD和白细胞破碎性血管炎；因此，固有免疫和获得性免疫似乎参与了IgG4-RD的发展。在IgG4-RD的发展过程中测量细胞因子和趋化因子似乎是有益的。值得注意的是，类固醇治疗对HT的有效性提示存在IgG4-RD相关的HT。因此，本报告突出了IgG4-RD的发病机制并提出了新的治疗机制。临床医生在HT的长期病程中应注意IgG4-RD和血管炎的发展。
