title: IgG4-related disease in the sinonasal cavity accompanied by intranasal structure loss.
abstract: IgG4-related disease was recently proposed under the classification of systemic chronic inflammatory disease. In the field of otolaryngology, organ-specific diagnostic criteria have been established for the occurrence of this condition in the salivary glands, but not in the sinonasal cavity. Here we report a case involving a 70-year-old man with IgG4-related disease in the sinonasal cavity. The patient, with the chief complaint of nasal bleeding, first visited a physician. However, the patient experienced recurrent bleeding with intranasal structure loss and was subsequently referred to our hospital. His IgG4 level was elevated, and histopathological examination of a tissue sample obtained from the edematous sphenoid sinus showed increased IgG4-positive plasma cells and storiform fibrosclerosis. A definitive diagnosis of IgG4-related rhinosinusitis was made on the basis of comprehensive criteria for IgG4-related disease. The disease showed a progressively destructive course that was clearly different from that of chronic sinusitis and represented a very rare case of IgG4-related rhinosinusitis. IgG4-related disease originating in the sinonasal cavity is rare, and, to the best of our knowledge, this is the first primary case of IgG4-related disease that originated in one side of the sinonasal cavity and showed progressive destruction.
authors.affiliation: Department of Otolaryngology, Toho University Omori Hospital, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Inoue
authors.affiliation: Department of Otolaryngology, Toho University Omori Hospital, Japan. Electronic address: wadakota@med.toho-u.ac.jp.
authors.forename: Kota
authors.initials: K
authors.lastname: Wada
authors.affiliation: Department of Otolaryngology, Toho University Omori Hospital, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Matsuura
authors.affiliation: Department of Otolaryngology, Toho University Omori Hospital, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Osafune
authors.affiliation: Department of Otolaryngology, Toho University Omori Hospital, Japan.
authors.forename: Yutaro
authors.initials: Y
authors.lastname: Ida
authors.affiliation: Department of Neurology, Keiyu Hospital, Japan.
authors.forename: Arifumi
authors.initials: A
authors.lastname: Kosakai
authors.affiliation: Department of Otolaryngology, Toho University Omori Hospital, Japan.
authors.forename: Hideo
authors.initials: H
authors.lastname: Edamatsu
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology; D004844:Epistaxis / Q000209:etiology; D005355:Fibrosis; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D012220:Rhinitis / Q000175:diagnosis* / Q000276:immunology; D012598:Sclerosis; D012852:Sinusitis / Q000175:diagnosis* / Q000276:immunology; D013101:Sphenoid Sinus / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D007074:Immunoglobulin G
keywords: Chronic rhinosinusitis (CRS); IgG4; IgG4-related disease; Sinonasal; Sinus