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Idiopathic Orbital Inflammation

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Orbital Inflammatory Diseases and Their Differential Diagnosis

Part of the book series: Essentials in Ophthalmology ((ESSENTIALS))

Abstract

Idiopathic orbital inflammation is an inflammatory process of the orbit and ocular adnexa without local or systemic etiology. It is the second or third most common orbital tumor. Idiopathic orbital inflammation can affect any race, age, and sex, with peak incidence at middle ages. It is usually a unilateral disease with almost equal involvement of the right and left orbits. Patients most commonly complain of pain (58–69 %) and diplopia (31–38 %) and present with periorbital swelling (75–79 %), proptosis (32–63 %), restriction in extraocular motility (54 %), and conjunctival hyperemia (48 %). It is classified as acute, subacute, and chronic based on the onset and duration of symptoms; dacryoadenitis, myositis, scleritis, perineuritis, and Tolosa-Hunt syndrome based on the involved tissue; and lymphocytic, granulomatous, sclerosing, and vasculitic inflammation based on pathologic features. Cytokines important in the induction of Th1 pathway are found to be increased in idiopathic orbital inflammation. This suggests the role of Th1 pathway in the pathogenesis. Toll-like receptors 2 are markedly expressed, supporting the role of innate immune system. The diagnosis is usually based on clinical and radiologic findings; however, biopsy is required to confirm the diagnosis. On computed tomography, it shows diffuse homogenous involvement of the orbital tissue. Histopathologic examination shows infiltrate of inflammatory cells of well-differentiated mature lymphocytes, admixed with plasma cells, neutrophils, and eosinophils. The management includes observation in asymptomatic patients and nonsteroidal anti-inflammatory agents in selected cases. Corticosteroids are the main treatment option with rapid and dramatic response. Immunosuppressive agents are used for patients who are unresponsive to corticosteroid or have a chronic, progressive course and require long-term treatment.

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Hakan Demirci declares that he has no conflict of interest.

No human or animal studies were carried out by the authors for this article.

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  1. Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, USA

    Hakan Demirci MD

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  1. Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, Ann Arbor, Michigan, USA

    Hakan Demirci

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Demirci, H. (2015). Idiopathic Orbital Inflammation. In: Demirci, H. (eds) Orbital Inflammatory Diseases and Their Differential Diagnosis. Essentials in Ophthalmology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-46528-8_2

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