스테로이드에 반응하지 않은 비강에 발생한 면역글로불린 G4 연관 경화성 질환 1례

• Published in: Journal of rhinology pp. 138 - 142
• Main Authors: 고석화, 윤형준, 정타오, 정진혁
• Format: Journal Article
• Language: Korean
• Published: 대한비과학회 01.11.2017
• Subjects: 이비인후과학
• ISSN: 1229-1498; 2384-4361
• DOI: 10.18787/jr.2017.24.2.138

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. KCI Citation Count: 1