Clinical outcomes and pathological characteristics of immunoglobulin G4-related ophthalmic disease versus orbital inflammatory pseudotumor

Abstract

Background/Aims: This study investigated the clinical and pathological features of immunoglobulin G(4)(IgG(4))-related ophthalmic disease. To clarify the features, we compared IgG(4)-related ophthalmic disease and orbital inflammatory pseudotumor. Methods: We retrospectively reviewed the medical records of 103 patients who were initially diagnosed with orbital inflammatory pseudotumor, and identified 16 cases in which the diagnosis was based on surgical biopsy and for which data in medical records were sufficient for analysis. Immunohistochemical staining of pathological specimens for IgG and IgG(4) was performed. Finally, six of IgG(4)-related ophthalmic disease patient and 10 of orbital inflammatory pseudotumor patient were analyzed. Results: The IgG(4)-related ophthalmic disease group had more IgG(4)-positive plasma cells and a higher IgG(4)/IgG plasma cell ratio than the orbital inflammatory pseudotumor group. Collagenous fibrosis and lacrimal gland involvement were significantly more frequent in the IgG(4)-related ophthalmic disease group. Dense lymphocyte infiltration, obliterative phlebitis, and bilateral lesions were more frequent in IgG(4)-related ophthalmic disease, but the differences were not significant. The recurrence-free period was shorter in the IgG(4)-related ophthalmic disease group (p = 0.035). Conclusions: The location of the lesion (lacrimal gland), count and ratio of IgG(4)-positive plasma cells, and collagenous fibrosis aid the diagnosis of IgG(4)-related ophthalmic disease in patients with idiopathic orbital mass-like lesions. In addition, maintenance therapy should be considered in patients with IgG(4)-related ophthalmic disease to prevent recurrence.