Understanding Igg4 Related Orbital Disease: A Rheumatologist's Perspective

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is also known as IgG4-related systemic disease, hyper-IgG4 disease, IgG4-related autoimmune disease. It is a multi-organ, fibro-inflammatory condition with tumefactive lesions of unknown etiology and characteristic histopathological features. It can affect all orbital tissues, including the lacrimal glands and extraocular muscles. A 52-year-old female patient, presented with complaints of swelling above her right eye. After undergoing a contrast-enhanced MRI, she had a biopsy. Consequently, the pathological findings were reported as consistent with IgG4-related sclerosing disease. The patient was initially prescribed corticosteroid therapy, followed by methotrexate and azathioprine treatment, which successfully stabilized her condition. Since the disease can affect almost all organs and systems, it is crucial for clinicians to have knowledge about early diagnosis and employ a multidisciplinary approach in patient management.