Immunoglobulin G4-Related Disease in The Head and Neck: Two Case Reports and Literature Review

Morteza MAZLOUM FARSI BAF1, Maryam SAHEBARI1, Kamran KHAZAENI2, Zahra REZAIEYAZDI1

1Rheumatic Diseases Research Center (RDRC), Ghaem Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran
2Sinus and Surgical Endoscopic Research Center, Ghaem Hospital, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

Keywords: Immunoglobulin G4-related disease, Mikulicz’s syndrome, Sjögren’s disease

Abstract

Immunoglobulin G4-related disease (IgG4-related disease) is a fibroinflammatory disorder defined by elevated serum IgG4 level and plasma cells tissue infiltration accompanied by fibrosis or sclerosis. In this article, we report two patients of IgG4 syndrome: a 60-year-old male with complaints of nasal congestion, rhinitis, anosmia and hearing loss, and a 28-year-old female with bilateral orbital and parotid gland swelling. Histopathological findings and high serum IgG4 level confirmed diagnosis of IgG4-related disease. Both patients were treated successfully with glucocorticoids and methotrexate. Herein, we also review clinical presentations of IgG4-related disease in head and neck, and related diagnostic and therapeutic approach.

Conflict of Interest

The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.

Financial Disclosure

The authors received no financial support for the research and/or authorship of this article.