title: Recent advances in knowledge regarding the head and neck manifestations of IgG4-related disease.
abstract: IgG4-related disease (IgG4-RD) is a chronic inflammatory disorder, characterized by elevated serum IgG4 levels as well as abundant infiltration of IgG4-positive plasmacytes and fibrosis in various organs, including the head and neck region. In particular, the salivary glands, orbit, and thyroid are common sites of disease involvement. IgG4-RD is diagnosed based on various clinical, serological, and histopathological findings, none of which are pathognomonic. Hence, various differential diagnoses, which exhibit elevated serum IgG4 levels and infiltration of IgG4-postive cells into tissues, need to be excluded, especially malignant diseases and mimicking disorders. Systemic corticosteroids are generally effective in inducing IgG4-RD remission; however, recurrent or refractory cases are common. In addition, although the pathogenic mechanisms of IgG4-RD remain unclear, an antigen-driven inflammatory condition is believed to be involved. Recent studies have indicated the important pathogenic role of B cell/T cell collaboration and innate immunity in this disease. Nevertheless, additional research and discussions are needed to resolve many remaining questions. In this review, we provide an overview of the recent insights on the history, clinical features, diagnosis, and treatment of IgG4-RD in the head and neck region. Furthermore, we have also addressed the pathogenesis of this disease.
authors.affiliation: Department of Otolaryngology, Sapporo Medical University School of Medicine, S1W16, Chuo-ku, Sapporo 060-8543, Japan. Electronic address: kent@sapmed.ac.jp.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Takano
authors.affiliation: Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, S1W16, Chuo-ku, Sapporo 060-8543, Japan.
authors.forename: Motohisa
authors.initials: M
authors.lastname: Yamamoto
authors.affiliation: Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine, S1W16, Chuo-ku, Sapporo 060-8543, Japan.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Takahashi
authors.affiliation: Department of Otolaryngology, Sapporo Medical University School of Medicine, S1W16, Chuo-ku, Sapporo 060-8543, Japan.
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Himi
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology*; D000069281:Autoimmune Hypophysitis / Q000188:drug therapy / Q000276:immunology; D003607:Dacryocystitis / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008199:Lymphadenitis / Q000188:drug therapy / Q000276:immunology; D008417:Mastoiditis / Q000188:drug therapy / Q000276:immunology; D009443:Neuritis / Q000188:drug therapy / Q000276:immunology; D010031:Otitis / Q000188:drug therapy / Q000276:immunology; D012220:Rhinitis / Q000188:drug therapy / Q000276:immunology*; D012793:Sialadenitis / Q000188:drug therapy / Q000276:immunology*; D012852:Sinusitis / Q000188:drug therapy / Q000276:immunology*; D013967:Thyroiditis, Autoimmune / Q000188:drug therapy / Q000276:immunology*
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G
keywords: Head and neck; IgG4; IgG4-related disease; Review; Salivary gland