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Review
. 2023 Jun 15;62(12):1849-1855.
doi: 10.2169/internalmedicine.0453-22. Epub 2022 Nov 9.

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

Tomoki Origuchi  1   2 , Tomohisa Uchida  1 , Tatsuki Sakaguchi  1 , Haruna Matsuo  1 , Toru Michitsuji  1 , Masataka Umeda  1 , Toshimasa Shimizu  1 , Tomohiro Koga  1 , Shin-Ya Kawashiri  1 , Naoki Iwamoto  1 , Kunihiro Ichinose  1 , Mami Tamai  1 , Masahiro Ichinose  3 , Koji Ando  3 , Ichiro Horie  4 , Nobuhiro Nakao  5 , Junji Irie  6 , Atsushi Kawakami  1
Affiliations
Review

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

Tomoki Origuchi et al. Intern Med. .

Abstract

We 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.

Keywords: IgG4-related disease; eosinophilia; hypereosinophilic syndrome.

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Figures

Figure 1.
Figure 1.
PET-CT findings. PET/CT showed a high accumulation of FDG in his left submandibular gland.
Figure 2.
Figure 2.
Histopathological images of the submandibular gland tumor. The submandibular gland tumor specimen showed (A) infiltration of lymphocytes and plasma cells and storiform fibrosis [Hematoxylin and Eosin (H&E) staining ×100, LPF]; (B) infiltration of lymphocytes and plasma cells (H&E staining ×400, HPPF); (C) dense infiltration of IgG-positive plasma cells (IgG immune staining ×400, HPF); (D) 100 IgG4-positive plasma cells/HPF and a 90% IgG4/IgG ratio (IgG4 immune staining ×400, HPF). LPF: low-power field, HPF: high-power field
Figure 3.
Figure 3.
Summary of the clinical course of this patient. The serum IgG4 level and peripheral blood eosinophil count were high at resection of the submandibular gland. Although eosinophilia temporarily decreased after the first shoulder joint injection of corticosteroid, the IgG4 level was unchanged. The peripheral blood eosinophil count then increased to 29,400/mL. Although the eosinophil count temporarily decreased after the second shoulder joint injection, it increased again afterward. Finally, oral 30 mg/day PSL resulted in the significant improvement of eosinophilia and IgG4 elevation. PSL: prednisolone, IgG4: immunoglobulin G4, PSL: prednisolone
See this image and copyright information in PMC

References

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