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ISRN Rheumatology
Volume 2012 (2012), Article ID 412896, 5 pages
http://dx.doi.org/10.5402/2012/412896
Review Article

Orbital IgG4-Related Disease: Clinical Features and Diagnosis

1Department of Ophthalmology, National Hospital Organization Nagoya Medical Center, 4-1-1, Sannomaru, Naka-ku, Nagoya-shi, Aichi-ken 460-0001, Japan
2Department of Pathology, National Hospital Organization Nagoya Medical Center, 4-1-1, Sannomaru, Naka-ku, Nagoya-shi, Aichi-ken 460-0001, Japan

Received 9 May 2012; Accepted 29 May 2012

Academic Editors: A. Adebajo and Z. M. Sthoeger

Copyright © 2012 Toshinobu Kubota and Suzuko Moritani. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.