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IgG4-Related Ophthalmic Disease. Part I: Background and Pathology

McNab, Alan A. D.Med.Sc., F.R.A.N.Z.C.O.*†; McKelvie, Penny M.D., F.R.C.P.A.

Ophthalmic Plastic and Reconstructive Surgery: March/April 2015 - Volume 31 - Issue 2 - p 83–88
doi: 10.1097/IOP.0000000000000363
Major Review

Purpose: To review the current state of knowledge of IgG4-related ophthalmic disease (IgG4-ROD).

Methods: A review of the literature and personal experience of the authors.

Results: IgG4-related disease is a recently recognized fibroinflammatory disorder that may affect 1 or more organs. It is characterized by lymphoplasmacytic infiltrates with large numbers of IgG4 positive plasma cells, storiform fibrosis, obliterative phlebitis, and eosinophil infiltration as well as peripheral eosinophilia, and in some cases, elevated serum levels of IgG4. These features are not always seen, and the diagnosis should be made by integrating clinical, imaging, and histopathological data, with reference to recently defined diagnostic criteria. IgG4-ROD forms a significant proportion of what has previously been labeled “idiopathic orbital inflammation” or reactive lymphoid hyperplasia. Orbital disease may occur alone, at the same time as disease elsewhere, or metachronously with systemic disease. Although almost any ocular adnexal tissue may be affected, there are several more common recognizable patterns of IgG4-ROD: 1) sclerosing dacryoadenitis; 2) enlargement of orbital nerves (most commonly the infraorbital nerve) associated with orbital myositis and lacrimal gland disease, often in combination with paranasal sinus disease, eosinophilia, and systemic involvement; and 3) sclerosing orbital inflammation.

Conclusions: Patients presenting with orbital inflammatory lesions should have biopsies obtained whenever possible. The examining pathologist should routinely look for features of IgG4-ROD, and if found, the patient should be investigated for other organ involvement. Early treatment may prevent destructive changes in affected tissues.

IgG4-related disease is a recently recognized fibroinflammatory disorder characterized by tumefactions infiltrated with large numbers of IgG4-postive plasma cells. Almost any organ can be affected. Orbital disease, in various patterns, is relatively common.

*Orbital Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital, Melbourne, Australia; Centre for Eye Research Australia, University of Melbourne, Melbourne, Australia; and Department of Anatomical Pathology, St Vincent’s Hospital, Melbourne, Australia

Accepted for publication October 23, 2014.

The authors have no financial or conflict of interest to disclose.

Presented in part at the Oculofacial Subspecialty Day of the American Academy of Ophthalmology meeting on November 2013 in New Orleans, LA.

Address correspondence and reprint requests to Associate Professor Alan A. McNab, D.Med.Sc., F.R.A.N.Z.C.O., Suite 216, 100 Victoria Parade, East Melbourne 3002, Victoria, Australia. E-mail:

© 2015 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.