A novel immunopathological association of IgG4-RD and vasculitis with Hashimoto's thyroiditis. (1st March 2016)

• Record Type: Journal Article
• Authors: 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
• Is Part Of: Endocrinology, diabetes & metabolism case reports. (2016)
• Publication Date: 2016-03-01
• Subjects: Adult -- Geriatric -- Male -- Asian - Japanese -- Japan; Thyroid -- Immunology -- Thyroid -- Thyroxine (T4) -- Triiodothyronine (T3) -- TSH -- Thyroiditis -- Hashimoto's disease -- IgG4–related systemic disease -- Leukocytoclastic vasculitis -- Lymphadenopathy; Skin pigmentation – spotty -- Eyelid swelling -- Salivary gland swelling -- IgG4/IgG ratio -- Immunoglobulin G4 -- C-reactive protein -- FT4 -- TSH -- Thyroid antibodies -- Cytokines -- Chemokines -- Thyroid ultrasonography -- CT scan -- X-ray -- Histopathology -- Haematoxylin and eosin staining -- Biopsy -- Skin biopsy -- White blood cell differential count -- Haemoglobin -- C3 complement -- C4 complement -- Antinuclear antibody -- Levothyroxine -- Glucocorticoids -- Prednisolone; Dermatology; Insight into disease pathogenesis or mechanism of therapy -- March -- 2016; Endocrinology -- Periodicals; Diabetes -- Periodicals; Diabetes Mellitus; Endocrinology; Diabetes; Endocrinology; Case Reports; Periodicals; Periodicals; 616.4
• DOI: 10.1530/EDM-16-0004
• Languages: English
• ISSNs: 2052-0573

Abstract

Summary: 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. Learning points: 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.