DR. SUMMY BHATNAGAR

DR.RWITUJA THOMAS, DR. ASHOK KUMAR GROVER

Abstract

Orbital IgG4-related disease is characterized by IgG4-positive lymphoplasmacytic infiltrations in ocular adnexal tissues. The signs and symptoms include chronic noninflammatory lid swelling and proptosis. Patients may have elevated serum levels of IgG4. Orbital IgG4-related disease must be differentiated from idiopathic orbital inflammation and ocular adnexal marginal zone B-cell lymphoma. Orbital tissues are affected by IgG4-related conditions. Here, we describe the clinicopathological features, differential diagnosis, treatments and histopathological findings of orbital IgG4-related disease in 19 patients. 11 (58%) were males. 13 (68%) had proptosis, 10 (53%) had lid edema and one had conjunctival congestion. 14(73.6%) underwent serum IgG4 testing of which 8 had elevated levels. 10 (53%) were biopsy proven cases of IgG4 disease. Systemic steroid therapy decreases the size of the lesions, but relapse often occurs when systemic steroid therapy is discontinued.