Abstract
Orbital IgG4-related disease, which can occur in adults of any age, is characterized by IgG4-positive lymphoplasmacytic infiltrations in ocular adnexal tissues. The signs and symptoms include chronic noninflammatory lid swelling and proptosis. Patients often have a history of allergic disease and elevated serum levels of IgG4 and IgE as well as hypergammaglobulinemia. Orbital IgG4-related disease must be differentiated from idiopathic orbital inflammation and ocular adnexal marginal zone B-cell lymphoma to ensure appropriate and effective treatment. Systemic steroid therapy decreases the size of the lesions, but relapse often occurs when systemic steroid therapy is discontinued.
Highlights
IgG4-related diseases are systemic syndromes characterized by elevated serum levels of IgG4 and IgG4-positive lymphoplasmacytic infiltrative lesions in the body
Orbital IgG4-related disease can occur in men and women of any age, many patients have a history of allergic diseases such as asthma and allergic rhinitis
Ocular adnexal marginal zone B-cell lymphomas make up the majority of lymphomas arising from the ocular adnexa
Summary
IgG4-related diseases are systemic syndromes characterized by elevated serum levels of IgG4 and IgG4-positive lymphoplasmacytic infiltrative lesions in the body. Orbital tissues are affected by IgG4-related conditions. It was first observed that Mikulicz’s disease correlated with IgG4-related disease [1] and later determined that IgG4-related disease can occur in any ocular adnexal tissues [2,3,4,5]. We review the clinicopathological features, differential diagnosis, and treatments of orbital IgG4-related disease on the basis of a meta-analysis of 42 patients including 3 case series studies
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