J Rhinol.  2023 Mar;30(1):48-52. 10.18787/jr.2023.00008.

A Case of IgG4-Related Disease With Sinonasal Involvement Presenting With Decreased Visual Acuity

• Lee JE
• Hwang SH

• KMID: 2540859
• DOI: http://doi.org/10.18787/jr.2023.00008

Abstract

IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by IgG4-positive plasma cell and T lymphocyte infiltration of multiple organs. It commonly involves the pancreas, lacrimal glands, and salivary glands, and it has been rarely reported in the sinonasal cavity. We herein report the case of a 47-year-old male patient whose chief complaint was decreased visual acuity. A tumefactive mass was found on imaging studies, originating from the sinonasal cavity and invading the orbit, kidney, and meninges. The mass was resected through endoscopic sinus surgery and was pathologically confirmed to be IgG4-RD. The patient was treated with steroid therapy and showed clinical improvement.

Keyword

Immunoglobulin G; Immunoglobulin G4-Related Disease; Paranasal Sinuses; Glucocorticoid

Figure

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  Fig. 1. Extraocular movement. The patient shows normal extraocular muscle function.

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  Fig. 2. Initial brain computed tomography (CT) and magnetic resonance imaging (MRI). A: Non-contrast brain CT shows no definite abnormality. B: T1-weighted MRI shows isointense dural thickening (white arrowheads). C: T2-weighted MRI shows markedly hypointense dural thickening (white arrowheads). D: Contrast-enhanced T1-weighted MRI shows smooth, homogeneous pachymeningeal enhancement along both cerebral hemispheres (white arrowheads).

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  Fig. 3. Preoperative endoscopic findings of the nasal cavity. A: The right nasal cavity is filled with crust and purulent discharge. B: The left nasal cavity shows inferior turbinate hypertrophy. S, septum; MT, middle turbinate; IT, inferior turbinate.

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  Fig. 4. Pathologic findings of the sinonasal cavity. A: Low-power view of dense lymphoplasmacytic infiltrate and hyaline fibrosis (hematoxylin and eosin staining, ×40 magnification). B: High-power view of storiform fibrosis and lymphoplasmacytic infiltrate (hematoxylin and eosin staining, ×100 magnification). C: High-power IgG4 immunohistochemical stain shows an increased IgG4 cell count and increased IgG4/IgG ratio (immunohistochemical staining for IgG4, ×200 magnification).

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  Fig. 5. Contrast-enhanced abdominal computed tomography shows hypodense, hypovascular lesions in the right kidney, suggestive of IgG4-related kidney disease.

Reference

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