Journal List > J Rhinol > v.23(1) > 1044363

Lee, Lee, and Chung: Three Cases of IgG4-Related Sclerosing Disease in Nasal Cavity

Abstract

IgG4-related sclerosing disease (IgG4-RSD) is characterized by extensive IgG4-positive plasma cell and T-lymphocyte infiltration of various organs. However, the nasal cavity is an extremely rare site of involvement. The authors experienced three cases of igG4-RSD in the nasal cavity. A 54-year-old woman presented with a nasal cavity mass on endoscopic exam and computed tomography (CT) revealed not only a hypoenhancing mass in the right nasal cavity but also a pituitary gland macroadenoma. Endoscopic removal and endoscopic TSA were performed. A 20-year-old man diagnosed with chronic sinusitis, underwent endoscopic sinus surgery. A 26-year-old woman showed thickening of the nasal septum and cribriform plate, and an infiltrating soft tissue lesion in right the alveolar process on CT and an endoscopic biopsy was performed. Pathologic specimens of all cases showed IgG4-positive plasma cell infiltration, consistent with IgG4-RSD. Serum IgG4 levels were checked in two of the cases, and the results were within normal range. All cases were further treated with methylprednisolone and all showed clinical improvement.

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Fig. 1.
Contrast enhance axial CT image revealed a hypoenhancing right nasal cavity mass (A). T1 enhance coronal MR image shows macroadenoma in pituitary gland (B).
jr-23-44f1.tif
Fig. 2.
Right inferior turbinate mass shows chronic rhinitis with increased IgG4-positive plasma cells (up to 106/HPF). Hematoxilin & eosin stain ×400 (A); IgG4 immunohistochemical stain ×400 (B).
jr-23-44f2.tif
Fig. 3.
T1 enhance axial MR image after treatment. At 10 months after the surgery, shows recurrent mass in right nasal cavity (A). At 4 months after steroid treatment, shows significant resolution of the mass (B).
jr-23-44f3.tif
Fig. 4.
Contrast enhance coronal CT image (A) shows thickening of nasal septum extending to cribriform plate. (B) shows bilateral maxillary, ethmoidal sinusitis.
jr-23-44f4.tif
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