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Igg4-related disease of the middle ear in a pediatric patient – the first reported case

Filip Hergešić orcid id orcid.org/0000-0001-7053-0582 ; KB Sveti Duh, Klinika za otorinolaringologiju i kirurgiju glave i vrata, Sveti Duh 64 64, 10000 Zagreb, Hrvatska
Lucija Grbac ; Medicinski fakultet Sveučilišta u Zagrebu, Šalata 3b, 10000 Zagreb, Hrvatska
Jakov Ajduk ; KBC Sestre milosrdnice, Klinika za otorinolaringologiju i kirurgiju glave i vrata, Vinogradska 29, 10000 Zagreb, Hrvatska; Medicinski fakultet Sveučilišta u Zagrebu, Šalata 3b, 10000 Zagreb, Hrvatska


Puni tekst: engleski pdf 188 Kb

str. 40-40

preuzimanja: 71

citiraj


Sažetak

Objective: To present the first reported case of IgG4-related disease (IgG4-RD) of the middle ear in a pediatric
patient and to highlight diagnostic challenges and treatment options. Materials and methods: A 15-year-old
girl presented with unilateral ear fullness and conductive hearing loss. Otoscopic, audiological, radiological,
and histopathological examinations were performed, including immunohistochemical staining for IgG4.
Results: The initial diagnosis was nonspecific chronic otitis media. After two surgeries, soft tissue from the
tympanic cavity and mastoid was removed. Histology revealed dense plasmacytoid infiltration with fibrosis,
and immunohistochemistry showed more than 50 IgG4-positive plasma cells per high-power field and an
IgG4+/IgG ratio above 40%. Serum IgG4 levels were normal. MRI of the head, neck, thorax, and abdomen
excluded additional lesions. Following surgery and a six-month course of oral corticosteroids, symptoms
partially improved and no recurrence was observed during a one-year follow-up. Conclusion: IgG4-RD of the
middle ear is extremely rare and has not previously been described in children. As it may mimic chronic otitis
media, this diagnosis should be considered in atypical presentations. Early recognition enables targeted
surgical and immunosuppressive treatment, preventing potential complications.

Ključne riječi

Immunoglobulin G4-related disease; middle ear; pediatrics

Hrčak ID:

336319

URI

https://hrcak.srce.hr/336319

Datum izdavanja:

10.11.2025.

Posjeta: 174 *