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Indolent Laryngeal Mass Causing Progressive Dysphagia

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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C. IgG-4 related disease

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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Article Information

Corresponding Author: Alexander Rivero, MD, Department of Head & Neck Surgery, Kaiser Permanente, 3600 Broadway, Oakland, CA 94611 (alexander.rivero@kp.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.

References
1.
Kamisawa  T, Funata  N, Hayashi  Y,  et al.  A new clinicopathological entity of IgG4-related autoimmune disease.  J Gastroenterol. 2003;38(10):982-984. doi:10.1007/s00535-003-1175-yPubMedGoogle ScholarCrossref
2.
Bozzalla Cassione  E, Stone  JH.  IgG4-related disease.  Curr Opin Rheumatol. 2017;29(3):223-227. doi:10.1097/BOR.0000000000000383PubMedGoogle ScholarCrossref
3.
Johnston  J, Allen  JE.  IgG4-related disease in the head and neck.  Curr Opin Otolaryngol Head Neck Surg. 2018;26(6):403-408. doi:10.1097/MOO.0000000000000487PubMedGoogle ScholarCrossref
4.
Zen  Y, Nakanuma  Y.  IgG4-related disease: a cross-sectional study of 114 cases.  Am J Surg Pathol. 2010;34(12):1812-1819. doi:10.1097/PAS.0b013e3181f7266bGoogle ScholarCrossref
5.
Takano  K, Yamamoto  M, Takahashi  H, Himi  T.  Recent advances in knowledge regarding the head and neck manifestations of IgG4-related disease.  Auris Nasus Larynx. 2017;44(1):7-17. doi:10.1016/j.anl.2016.10.011PubMedGoogle ScholarCrossref
6.
Reder  L, Della-Torre  E, Stone  JH, Mori  M, Song  P.  Clinical manifestations of IgG4-related disease in the pharynx: case series and review of the literature.  Ann Otol Rhinol Laryngol. 2015;124(3):173-178. doi:10.1177/0003489414549574PubMedGoogle ScholarCrossref
7.
Deshpande  V, Zen  Y, Chan  JK,  et al.  Consensus statement on the pathology of IgG4-related disease.  Mod Pathol. 2012;25(9):1181-1192. doi:10.1038/modpathol.2012.72PubMedGoogle ScholarCrossref
8.
Mulholland  GB, Jeffery  CC, Satija  P, Côté  DWJ.  Immunoglobulin G4-related diseases in the head and neck: a systematic review.  J Otolaryngol Head Neck Surg. 2015;44:24. doi:10.1186/s40463-015-0071-9PubMedGoogle ScholarCrossref
9.
Thompson  A, Whyte  A.  Imaging of IgG4-related disease of the head and neck.  Clin Radiol. 2018;73(1):106-120. doi:10.1016/j.crad.2017.04.004PubMedGoogle ScholarCrossref
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