J Rhinol.  2016 May;23(1):44-48. 10.18787/jr.2016.23.1.44.

Three Cases of IgG4-Related Sclerosing Disease in Nasal Cavity

Affiliations
  • 1Department of Otoloaryngology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. yschung@amc.seoul.kr

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.

Keyword

IgG4-related sclerosing disease

MeSH Terms

Adult
Alveolar Process
Biopsy
Ethmoid Bone
Female
Humans
Immunoglobulin G
Methylprednisolone
Middle Aged
Nasal Cavity*
Nasal Septum
Pituitary Gland
Plasma Cells
Reference Values
Sinusitis
T-Lymphocytes
Young Adult
Immunoglobulin G
Methylprednisolone

Figure

  • 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).

  • 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).

  • 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).

  • Fig. 4. Contrast enhance coronal CT image (A) shows thickening of nasal septum extending to cribriform plate. (B) shows bilateral maxillary, ethmoidal sinusitis.


Cited by  3 articles

A Case of Immunoglobulin G4-Related Sclerosing Disease of the Paranasal Sinus Mimicking Nasal Malignancy
Juyong Chung, Jae Hoon Lee
J Rhinol. 2017;24(1):60-64.    doi: 10.18787/jr.2017.24.1.60.

A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity
Seok Hwa Ko, Hyung Joon Yoon, Tao Zheng, Jin Hyeok Jeong
J Rhinol. 2017;24(2):138-142.    doi: 10.18787/jr.2017.24.2.138.

A Case of IgG4-Related Disease With Sino-Nasal Involvement
Young Seok Han, Jun Sang Cha, Min Jung Jung, Joo Yeon Kim
Korean J Otorhinolaryngol-Head Neck Surg. 2022;65(11):705-712.    doi: 10.3342/kjorl-hns.2021.00598.


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