Management of IgG4-related Sclerosing Disease

Abstract

IgG4-related sclerosing disease is a systemic syndrome that is distinguished by high levels of both IgG4 and IgG4-positive lymphoplasmocytic infiltrative lesions in the body. We report an occurrence of orbital IgG4-related disease in a 62-year-old Hispanic male. IgG4 related sclerosing disease involving the orbit affects patients ranged from ages 30 to 86 with a 1:1 male to female ratio (1,2). The patient presented with bilateral painless, progressive nodular lesions of the lacrimal glands. Pathologic evaluation from the biopsy of the orbital mass showed an increase in IgG4 positive plasma cells, fibrosclerosis, and lymphoid hyperplasia. The patient was treated with a high dose of oral prednisone for a period of 3 months and was then placed on a maintenance dose with rapid resolution of both orbital masses. The work-up, diagnostic tools, and treatment of IgG4 disease are discussed. It is important to note that IgG4 can affect all orbital structures and physical findings, lab results, and biopsy results are required for appropriate diagnosis and management.