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KMID : 0900220170240020138
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대한비과학회지
2017 Volume.24 No. 2 p.138 ~ p.142
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A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity
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Ko Seok-Hwa
Yoon Hyung-Joon
Zheng Tao
Jeong Jin-Hyeok
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Abstract
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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 IgG4related sclerosing disease treated with azathioprine for 2 years along with a related literature review.
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KEYWORD
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IgG4-related sclerosing disease, Plasma cell, Nasal cavity, Paranasal sinus
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