Since clarification of the relationship between Mikulicz’s disease and IgG4-related disease (IgG4-RD), basic research and clinical work on IgG4-RD in ophthalmology have progressed considerably, particularly with regard to the lacrimal gland lesions. Although it is clear that the lacrimal gland is the most common of the various orbital structures to be involved by IgG4-RD, other orbital structures are also involved with a high frequency, particularly the extraocular muscles and infraorbital nerve. Moreover, IgG4-related lesions may extend from the orbit to affect adjacent structures in the orbital apex, the lacrimal drainage system, and the eyeball. “IgG4-related ophthalmic disease” has been proposed as an appropriate term that encompasses disease involvement at all of these sites. Among inflammatory and lymphoproliferative disorders of the orbit, approximately one fourth appear to be associated with IgG4-RD.
KeywordsPleomorphic Adenoma Lacrimal Gland Malt Lymphoma Extraocular Muscle Infraorbital Nerve
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