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December 2018

Pediatric Laryngeal Expression and Surgical Treatment of IgG4-Related Disease

Author Affiliations
  • 1Department of Otolaryngology – Head and Neck Surgery, University of Minnesota, Minneapolis
  • 2Department of Pathology, Mayo Clinic, Rochester, Minnesota
  • 3Department of Otorhinolaryngology – Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota
  • 4ENT and Facial Plastic Surgery, Children's Minnesota, Children's Specialty Center, Minneapolis, Minnesota
JAMA Otolaryngol Head Neck Surg. 2018;144(12):1183-1184. doi:10.1001/jamaoto.2018.2258

IgG4-related disease is a relatively unknown and newly described immunologic disease involving chronic relapsing sclerosis of a variety of organ systems.1 This disease remains underrecognized owing to its slow progression, nonspecific radiologic findings, and rarity.

This disease mainly affects adults, with equal sex distribution.1,2 It presents insidiously with tumefaction of the involved organ(s), most commonly exocrine organs. Cause and pathogenesis are unknown. Incidence is less than 1 in 100 000 in adults and incidence in children is limited to case reports.2 Radiographic findings are nonspecific and show diffuse organ enlargement, homogenous contrast enhancement, and minimally enlarged lymphadenopathy.3

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