IgG4-related Disease Masquerading as Recurrent Scleritis and Chronic Conjunctivitis

Purpose: To report atypical ophthalmologic manifestations and complications of IgG4-related disease (IgG4-RD). Methods: Patients with isolated ophthalmologic involvement of IgG4-RD other than lacrimal or orbital infiltration seen between 2009 and 2011 in a single tertiary center were retrospectively reviewed and their clinical and histological features, treatment, and prognosis were studied. Case reports: Two patients (mean age 56.5 years) were included. One patient presented with recurrent anterior and posterior scleritis, and one patient had chronic conjunctival infiltration. Histopathology demonstrated lymphoplasmacytic proliferation with overexpression of IgG4(+) plasma cells. Both patients initially responded to a high dose of oral corticosteroids (1 mg/kg/d). However, one patient required the adjunction of methotrexate and one patient developed an intra-epithelial conjunctival carcinoma on the site of the initial lesion. Conclusion: Patients with atypical presentation of IgG4-RD, such as chronic conjunctival infiltration or scleritis, can suffer from considerable diagnostic delay leading to fibrosis or malignancy development. We report the first case of conjunctival carcinoma in a patient with IgG4-RD.