title: A novel immunopathological association of IgG4-RD and vasculitis with Hashimoto's thyroiditis.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: Hiroto
authors.initials: H
authors.lastname: Minamino
authors.affiliation: The First Department of Medicine, Wakayama Medical University , 811-1, Kimiidera, Wakayama, 641-8509 , Japan.
authors.forename: Hidefumi
authors.initials: H
authors.lastname: Inaba
authors.affiliation: The First Department of Medicine, Wakayama Medical University , 811-1, Kimiidera, Wakayama, 641-8509 , Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ariyasu
authors.affiliation: The First Department of Medicine, Wakayama Medical University , 811-1, Kimiidera, Wakayama, 641-8509 , Japan.
authors.forename: Hiroto
authors.initials: H
authors.lastname: Furuta
authors.affiliation: The First Department of Medicine, Wakayama Medical University , 811-1, Kimiidera, Wakayama, 641-8509 , Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Nishi
authors.affiliation: Department of Dermatology, Wakayama Medical University , Wakayama , Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yoshimasu
authors.affiliation: Department of Hematology, Wakayama Medical University , Wakayama , Japan.
authors.forename: Akinori
authors.initials: A
authors.lastname: Nishikawa
authors.affiliation: Department of Respiratory Medicine & Medical Oncology, Wakayama Medical University , Wakayama , Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Nakanishi
authors.affiliation: Department of Otolaryngology, Wakayama Medical University , Wakayama , Japan.
authors.forename: Shigeki
authors.initials: S
authors.lastname: Tsuchihashi
authors.affiliation: Department of Human Pathology, Wakayama Medical University , Wakayama , Japan.
authors.forename: Fumiyoshi
authors.initials: F
authors.lastname: Kojima
authors.affiliation: Department of Human Pathology, Wakayama Medical University , Wakayama , Japan.
authors.forename: Shin-Ichi
authors.initials: S
authors.lastname: Murata
authors.affiliation: Department of Diabetes and Endocrinology, Wakayama Red Cross Hospital , Wakayama , Japan.
authors.forename: Gen
authors.initials: G
authors.lastname: Inoue
authors.affiliation: The First Department of Medicine, Wakayama Medical University , 811-1, Kimiidera, Wakayama, 641-8509 , Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Akamizu