• Internal medicine · Jun 2023

    Review Case Reports

    Immunoglobulin G4-related disease with Marked Eosinophilia: A Case Report and Literature Review.

    • Tomoki Origuchi, Tomohisa Uchida, Tatsuki Sakaguchi, Haruna Matsuo, Toru Michitsuji, Masataka Umeda, Toshimasa Shimizu, Tomohiro Koga, Shin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Masahiro Ichinose, Koji Ando, Ichiro Horie, Nobuhiro Nakao, Junji Irie, and Atsushi Kawakami.
    • Department of Immunology and Rheumatology, Unit of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan.
    • Intern. Med. 2023 Jun 15; 62 (12): 184918551849-1855.

    AbstractWe encountered a 78-year-old Japanese man with IgG4-related sialoadenitis complicated with marked eosinophilia. We diagnosed him with IgG4-RD (related disease) with a submandibular gland tumor, serum IgG4 elevation, IgG4-positive plasma cell infiltration, and storiform fibrosis. During follow-up after total incision of the submandibular gland, the peripheral eosinophil count was markedly elevated to 29,480/μL. The differential diagnosis of severe eosinophilia without IgG4-RD was excluded. The patient exhibited a prompt response to corticosteroid therapy. His peripheral blood eosinophil count was the highest ever reported among similar cases. We also review previous cases of IgG4-RD with severe eosinophilia.

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