Clinical Pathologic ReviewsIgG4-Related Systemic Disease as a Cause of “Idiopathic” Orbital Inflammation, Including Orbital Myositis, and Trigeminal Nerve Involvement
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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