J Rhinol.  2017 Nov;24(2):138-142. 10.18787/jr.2017.24.2.138.

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

• Ko SH
• Yoon HJ
• Zheng T
• Jeong JH

• KMID: 2398834
• DOI: http://doi.org/10.18787/jr.2017.24.2.138

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.

Keyword

IgG4-related sclerosing disease; Plasma cell; Nasal cavity; Paranasal sinus

MeSH Terms

Autoimmune Diseases
Azathioprine
Bile Ducts
Biopsy
Diagnosis
Fibrosis
Humans
Immunoglobulins*
Lung
Nasal Cavity*
Pancreas
Pancreatitis
Plasma Cells
Rituximab
Salivary Glands
Urinary Bladder
Azathioprine
Immunoglobulins
Rituximab

Figure

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  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.

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  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).

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  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).

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  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).

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