Elsevier

Survey of Ophthalmology

Volume 57, Issue 1, 2 January 2012, Pages 26-33
Survey of Ophthalmology

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

https://doi.org/10.1016/j.survophthal.2011.07.004Get rights and content

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.

Section snippets

Case Presentation

A 56-year-old man with a history of gout, asthma, and infertility secondary to diethylstilbestrol exposure in utero presented with a 30-year history of recurrent, non-painful proptosis and chemosis of his orbits, left greater than right (Fig. 1A). The initial episode, which occurred in 1981, was acute and severe. Over a period of days, the patient was unable to open his left eye. Since that time, he had experienced recurrences approximately three to four times per year, usually in the left eye,

Case Discussion

IgG4-RD is an inflammatory process of unclear etiology that is increasingly recognized as a cause of tumefactive lesions in multiple tissues and organs, including pancreas, submandibular and parotid glands, biliary tree, thyroid, retroperitoneum, kidneys, and lungs.8, 10 This diversity of organ system involvement is unified by a consistent histopathology characterized by lymphoplasmacytic inflammation, phlebitis, and varying degrees of fibrosis that stains for a disproportionate number of

Conclusion

In our case the decision to pursue a tissue diagnosis after 30 years of empiric therapy led to a specific diagnosis and a targeted treatment. IgG4-RD can cause IOI, including orbital myositis, that may be exquisitely sensitive to rituximab, offering an important new therapeutic option. The availability of such new treatments underscores the importance of histological diagnosis obtained from biopsy. Also, treatment with prednisone may confound the diagnosis by temporarily normalizing the serum

Method of Literature Search

The authors conducted Medline and PubMed literature searches in English. Search words included IgG4-related systemic disease, IgG4, idiopathic orbital inflammatory disease, orbital pseudotumor, rituximab, lacrimal gland, dacryoadenitis, orbital myositis, wegener's granulomatosis, sarcoidosis, orbital lymphoma, grave's disease, trigeminal nerve, and thyroid orbitopathy, in various combinations. Articles were reviewed critically and we included those that helped us better understand and

Disclosure

The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article.

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