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Irfan Khan, Steven A Newman, Maria Kirzhner; Idiopathic Orbital Inflammation: A University of Virginia Perspective. Invest. Ophthalmol. Vis. Sci. 2014;55(13):4083.
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To review cases of non-infectious idiopathic orbital inflammation (IOI) evaluated at the University of Virginia in order to further characterize its clinical features, as well as treatment outcomes.
A retrospective chart review was performed by identifying individuals evaluated at the UVA Department of Ophthalmology over the past 3 years with diagnosis codes for IOI.
Forty one patients were identified. There were 24 females and 17 males with an average age of 47.5 years (range 20-74). Follow up ranged from 4 weeks to 16 years. Pain, proptosis and periocular swelling were the most common symptoms, followed by diplopia. 48% of patients in this series had radiographic evidence of inflammation of multiple orbital structures as opposed to isolated myositis/dacryoadenitis. 5 had inflammation of the orbital apex. All patients were evaluated with CT, and 44% underwent MRI. 33% of patients underwent biopsy. The patient’s who underwent biopsy generally had an incomplete response to therapy with steroids. Additionally, all but one of the patients that presented without pain underwent biopsy. 78% of patient’s who underwent biopsy demonstrated IgG 4+ staining on histology. 93% of the patient’s were treated with corticosteroids as initial therapy. 1 patient was treated with radiation after poor response to steroids and 1 patient was treated with surgical debulking. 7 patients were secondarily treated with biologic agents (Rituximab). Only 9 patients experienced complete resolution of symptoms. Residual motility deficits/proptosis were seen in the majority of patients.
In conclusion, as noted in previous studies, pain and proptosis were the most common symptoms of IOI. In patients who are suspected of having idiopathic orbital inflammation, but fail to respond to corticosteroids, or have atypical symptoms (lack of pain), biopsy should be considered as those who demonstrate a sclerosing IgG 4+ histology may be treated successfully with biologic agents. Review of this series also demonstrated that the incomplete resolution of symptoms was noted in the majority of patients with IOI.
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