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A 29-year-old man with a history of reactive airway disease and eczema presented to the clinic with progressive odynophagia, hoarseness, and dysphagia of solids, liquids, and secretions for the past 6 months. There were no respiratory symptoms, and results of a complete head and neck physical examination were unremarkable. Flexible nasolaryngoscopy was performed and revealed erythema and swelling to the right arytenoid. A 2-week course of doxycycline was completed for presumed infectious supraglottitis without a change in symptoms. A biopsy and culture of the lesion was then collected in the office and revealed inflammatory disease but ultimately was nondiagnostic. The patient chose observation vs further workup at that time.
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The patient was diagnosed with IgG4-related disease (IgG4-RD), which is a relatively new diagnosis that was first discussed as a unique clinical syndrome in 2003 involving the pancreas.1 The disease has since been characterized as a chronic fibroinflammatory condition with tumefactive lesions that can affect nearly every organ in the body; the exact pathogenesis is not completely understood.2,3 Presentation of the disease will most often occur in an elderly patient with a nonpainful mass.3 In 2010, Zen et al4 performed a cross-sectional analysis and identified the head and neck region as the third most common presentation of IgG4-RD; only systemic and pancreatic hepatobiliary presentations were more prevalent in their study.5 A diagnosis of IgG4-RD has been described within the head and neck region in the salivary glands, lacrimal glands, orbit, sinonasal region, thyroid, pituitary gland, ear, lymph nodes, and less commonly the pharynx.5
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Alexander Rivero, MD, Department of Head & Neck Surgery, Kaiser Permanente, 3600 Broadway, Oakland, CA 94611 (firstname.lastname@example.org).
Published Online: December 5, 2019. doi:10.1001/jamaoto.2019.3732
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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