IgG4-Related Systemic Disease as a Cause of “Idiopathic” Orbital Inflammation, Including Orbital Myositis, and Trigeminal Nerve Involvement

Abstract IgG4-related systemic disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the find...

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Published in:	Survey of ophthalmology 2012-01, Vol.57 (1), p.26-33
Main Authors:	Wallace, Zachary S., MD, Khosroshahi, Arezou, MD, Jakobiec, Frederick A., MD, Deshpande, Vikram, MD, Hatton, Mark P., MD, Ritter, Jill, MD, Ferry, Judith A., MD, Stone, John H., MD, MPH
Format:	Article
Language:	eng
Subjects:	Antibodies, Monoclonal, Murine-Derived - therapeutic use Biological and medical sciences dacryoadenitis Diagnosis, Differential Diseases of striated muscles. Neuromuscular diseases Exophthalmos - pathology Glucocorticoids - therapeutic use Humans Hypergammaglobulinemia - drug therapy Hypergammaglobulinemia - immunology Hypergammaglobulinemia - pathology Idiopathic Inflammation IgG4-related systemic disease Immunoglobulin G - blood Immunologic Factors - therapeutic use Male Medical sciences Middle Aged Miscellaneous myositis Neurology Ophthalmology Orbital Myositis - drug therapy Orbital Myositis - immunology orbital pseudotumor Orbital Pseudotumor - drug therapy Orbital Pseudotumor - immunology Orbital Pseudotumor - pathology Prednisone - therapeutic use Rituximab Tomography, X-Ray Computed Trigeminal Nerve Diseases - drug therapy Trigeminal Nerve Diseases - immunology Trigeminal Nerve Diseases - pathology
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Summary:	Abstract IgG4-related systemic disease (IgG4-RD) is an inflammatory condition of unknown etiology that has been identified as the cause of tumefactive lesions in a number of tissues and organs. The role of the IgG4 remains to be clarified fully, but the histopathologic diagnosis hinges upon the finding of IgG4-bearing plasma cells in addition to characteristic morphologic features, with or without elevated seum IgG4. We present a 56-year-old man with orbital pseudotumor in whom, after 30 years of intractable disease, biopsy showed IgG4-RD involving the lacrimal gland, extraocular muscles, intraconal fat, and trigeminal nerve. Six months after initiating treatment with rituximab, his disease remained dormant, with improvement in his proptosis and normalization of serum IgG4 levels. We review the differential of idiopathic orbital inflammatory disease, including IgG4-RD, and emphasize the need for biopsy for accurate diagnosis and to guide appropriate treatment.
ISSN:	0039-6257 1879-3304