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

doi:10.18787/jr.2017.24.2.138

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Ko, Yoon, Zheng, and Jeong: A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity

Case Report

Journal of Rhinology 2017; 24(2): 138-142.

Published online: 30 November 2017

DOI: https://doi.org/10.18787/jr.2017.24.2.138

A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity

Seok Hwa Ko, MD, Hyung Joon Yoon, MD, Tao Zheng, MD, Jin Hyeok Jeong, MD, PhD

Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Korea.

Corresponding author (ent@hanyang.ac.kr)

Received 13 March 2017 Revised 6 April 2017 Accepted 22 April 2017

Journal of Rhinology

Abstract

IgG4-related sclerosing disease was first discovered when researching autoimmune pancreatitis. It is an autoimmune disease that causes fibrosis on the pancreas, bile duct, gall bladder, salivary glands, and lungs. It is rarely reported in the nasal cavity. The diagnosis of IgG4-related sclerosing disease is based on biopsy findings demonstrating the characteristic histopathologic findings and immunohistochemical staining. IgG4-related sclerosing disease shows good response to high-dose steroid therapy. However, some patients do not respond to this treatment. Azathioprine or rituximab can be helpful in such case. We report our experience with IgG4-related sclerosing disease treated with azathioprine for 2 years, along with a related literature review.

Keywords: IgG4-related sclerosing disease, Plasma cell, Nasal cavity, Paranasal sinus

Figures and Tables

Fig. 1

Endoscopic findings of nasal cavity (2014.11.13). A: Large sized perforation (arrow) with very thin mucosal membrane and severe inflammation in right nasal cavity after crust removal. B: Left Nasal cavity before crust removal. White material is a Merocel packing to protect blood aspiration during deep biopsy. C: Ulcerative lesion (arrow) around left lateral nasal wall. U: Uncinate process, M: Middle turbinate, S: Septum.

Fig. 2

Radiologic findings. CT image showing mucosal thickening of left maxillary sinus, Coronal view (A). Axial view (B). Coronal MR image showing mucosal thickening of supero-anterior portion of left lateral nasal wall with left maxillary mucosal thickening, T1-weighted image (C). T1-weighted with gadolinium enhancement (D). T2-weighted image (E).

Fig. 3

Pathologic findings. It reveals fibrosclerotic change with marked lymphoplasmacytic inflammation (H&E, ×400) (A). It shows large Immunoglobulin G4 (IgG4)-positive plasma cell in fibrosclerotic stroma, measuring 35/HPF and the ratio of IgG4 with IgG is about 40% (IgG4 immunoperoxidase stain, ×400) (B).

Fig. 4

Endoscopic findings during treatment. Septal perforation with crust and ulcerative mucosal lesion was seen during treatment (After using azathioprine 1month, 14.3.31) (A). Well healed mucosa of nasal septum was seen after stop medication (16.2.14) (B).

Table 1

Treatment protocol and laboratory finding during follow-up

PDS: Prednisolone, MTX: Methotrexate, AZA: Azathioprine

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