Immunoglobulin G4-related ophthalmic disease presenting as uveitis

This report documents a 47-year-old man who presented with back pain, uveitis and an elevated Westergren sedimentation rate. On biopsy, a paraspinal lesion showed a nonspecific chronic inflammatory cell infiltrate. The eye symptoms, after initially responding to immunosuppressive therapy, worsened and progressed to pain, resulting in an extirpation of the right eye. The histopathology of the excised eye showed an inflammatory pseudotumor marked by a lymphoplasmacytic infiltrate, areas of fibrosis, rare evidence of obliterative phlebitis and, focally, over 20 Immunoglobulin G4 (IgG4)-positive staining cells per high power microscopic field. IgG4-related ophthalmic disease is a relatively rare inflammatory lesion involving the eye and periorbital region. It is defined by a marked lymphoplasmacytic cell infiltrate, fibrosis obliterative phlebitis and increased IgG4 immunostaining (at least 10 cells per high power microscopic field in excised tissue). The entity is not unique to the eye, and has been described in other organs including the brain, endocrine organs, liver and kidney. The clinical presentation is often related to the location of the inflammatory infiltrates, and treatment involves the use of corticosteroids and other immunosuppressive agents. It is important to recognize IgG4-related ophthalmic disease because the condition appears to put patients at increased risk of developing lymphoma.