Ege Klinikleri Tıp Dergisi, cilt.63, sa.3, ss.227-231, 2025 (TRDizin)
Abstract Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by dense lymphoplasmacytic infiltration, storiform fibrosis, and increased IgG4-positive plasma cells. It can affect virtually any organ, including the pancreas, salivary glands, lymph nodes, and orbital tissues. The ophthalmic manifestation, IgG4-related ophthalmic disease (IgG4-ROD), typically involves the lacrimal glands and orbital soft tissues, often presenting with eyelid swelling, proptosis, or dry eye symptoms. Diagnosis requires a comprehensive approach incorporating clinical findings, serologic markers, imaging studies, and histopathological confirmation. Moreover, emerging evidence suggests an increased risk of malignancy associated with IgG4-RD, highlighting the need for thorough systemic evaluation and long-term surveillance. This report describes a 63-year-old female patient with bilateral eyelid swelling and dry eye symptoms, elevated serum IgG4 and characteristic orbital imaging findings. Histopathological confirmation was obtained from pancreatic tissue, and systemic evaluation revealed concurrent breast and lung malignancies managed surgically, with clinical stability achieved under low-dose corticosteroids. Keywords: eyelid swelling, IgG4-related disease, IgG4-related ophthalmic disease, malignancy