Recurrent Submandibular Mass and Bilateral Periorbital Edema in IgG4-Related Disease: A Case Report

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated systemic disorder characterized by inflammatory, proliferative, and fibrotic lesions that can affect any body organ. The severity and extent of organ involvement vary significantly among individuals. Diagnosis of IgG4-RD necessitates a comprehensive assessment, including clinical presentation, radiological findings, laboratory tests, and histopathological examination. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria offer a valuable framework for diagnosing IgG4-RD. Although glucocorticoids and rituximab are the primary treatment options, the optimal long-term management strategy for IgG4-RD remains undetermined.