Purpose of review To summarize current evidence regarding the pathophysiology, diagnostic criteria, and management of IgG4 disease in the head and neck. Recent findings The anti-CD20 antibody, rituximab is being used increasingly as a primary treatment modality in cases with a definitive diagnosis. Despite the favorable prognosis, it is now recognized that the incidence of cancer development within 3 years of diagnosis is higher than that of the general population. Summary IgG4-related disease is a sclerosing, chronic inflammatory disease, that is benign, and usually occurs in middle-aged to elderly individuals. Definitive diagnosis of IgG4-related disease requires radiological confirmation of enlargement of the affected organ, elevated serum IgG4 levels, and characteristic histopathological findings. Treatment is primarily with systemic corticosteroids or the monoclonal anti-CD20 antibody rituximab, surgical excision, or a combination of treatment modalities. Prognosis is good with complete remission in up to 90% of patients. Correspondence to James Johnston, Department of Surgery, University of Auckland, PO Box 99743, Newmarket, Auckland 1149, New Zealand. Tel: +64 21 1716814; e-mail: jj.johnston@auckland.ac.nz Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.
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