IgG4-related Ophthalmic Disease in Idiopathic Sclerosing and Non-Sclerosing Orbital Inflammation: A 25-Year Experience

Purpose: To determine the prevalence, clinical manifestations, and treatment outcomes of IgG4-related ophthalmic disease (IgG4-ROD) in previously diagnosed idiopathic orbital inflammation (IOI). Materials and Methods: Thirty one surgically treated patients with IOI between 1990 and 2015 were divided into sclerosing (SIOI) and non-sclerosing (NSIOI) to identify IgG4-ROD. Preserved pathological specimens were immunostained for IgG4 cells. Changes in clinical features were evaluated before and after treatment. Results: Out of the 31 patients, 15 (48.4%) had IgG4-ROD, consisting of 7 of the 16 SIOI (43.8%) and 8 of the 15 NSIOI patients (53.3%). Among 15 patients with IgG4-ROD, 4 (26.7%) achieved complete remission, which was significantly less than among IgG4-unrelated patients (11/16, 68.8%, p = .03). Furthermore, 3 out of 15 IgG4-ROD patients (20%) experienced recurrence, all of whom had SIOI, compared to 0% among IgG4-unrelated patients (p = .1). Conclusions: IgG4-ROD is common among previously identified IOI in our study. Treatment response is modest in IgG4-ROD patients.