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
Jordan VA, Herrera Hernandez LP, Cofer SA, Roby BB. Pediatric Laryngeal Expression and Surgical Treatment of IgG4-Related Disease. JAMA Otolaryngol Head Neck Surg. 2018;144(12):1183–1184. doi:10.1001/jamaoto.2018.2258
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