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

Affiliations
  • 1Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Korea. ent@hanyang.ac.kr

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
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