Akademik Veri Yönetim Sistemi
Saudi Journal of Otorhinolaryngology Head and Neck Surgery, cilt.27, sa.4, 2025 (Hakemli Dergi)
Isolated Orbital Involvement of IgG4-Related Disease Mimicking Malignancy
Abstract
Immunoglobulin G4‑related disease (IgG4‑RD) is a rare, immune‑mediated systemic condition
that can affect the orbit. In this report, we describe a 76‑year‑old female patient who experienced
a 4‑month history of pain in the right orbit and vision loss. Imaging studies revealed a mass
occupying the right orbital apex. Although the patient’s serum IgG4 level was within normal
limits, which complicated the diagnostic process, histopathological evaluation revealed significant
tissue infiltration by IgG4‑positive plasma cells, confirming the diagnosis of IgG4‑RD. Following
treatment with azathioprine and methylprednisolone, the lesion regressed; however, the vision loss
became permanent. Isolated orbital involvement in IgG4‑RD is rare and can mimic neoplasms.
Early diagnosis and combination therapy may help prevent disease progression, although complete
functional recovery is not always possible.
Keywords: Immunoglobulin G4‑related disease, immunosuppressive treatment, orbit, vision loss