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Orbital and Adnexal Sarcoidosis

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Orbital Inflammatory Diseases and Their Differential Diagnosis

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

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Abstract

Sarcoidosis is an idiopathic, noncaseating granulomatous inflammatory disorder. It can affect any race, gender, and age, with a peak incidence between the ages of 20 and 39 years. Sarcoidosis has multifactorial pathogenesis. Among siblings, all first-degree and second-degree relatives of sarcoidosis patients, the risk of developing disease increased fivefold. The genetic basis of sarcoidosis involves the class 2 HLA genes. HLA-DRB1*0401 is associated with an increased risk of ocular involvement. The presence of mycobacterial DNA in PCR studies and the mycobacterial catalase-peroxidase protein in mass spectrometry of sarcoidosis tissue suggested the role of mycobacterial organisms in pathogenesis. Additionally, serum amyloid A (SAA) was expressed higher in sarcoidosis and induced granulomatous lung inflammation in animal models. Eye and skin involvements are the most common extrathoracic presentations. Ocular manifestations are anterior uveitis (73 %), vitritis (62 %), retinal and choroidal lesions (34 %), and ocular adnexal and orbital lesions (10 %). In the orbital and ocular adnexa, the lacrimal gland (42 %) is most commonly affected, followed by the orbital tissue (39 %), eyelid (12 %), and lacrimal sac (8 %). Diagnosis is based on clinical and radiologic findings. On imaging, homogenous or lobular enlargement of the lacrimal gland or diffuse homogenous involvement of orbital tissue is observed. Noncaseating inflammation is the main histopathological finding. Management involves observation in asymptomatic patients, while corticosteroid and steroid-sparing immunosuppressive agents are the mainstay of treatment. Periocular corticosteroid and antitumor necrosis factor alpha agent infliximab have been used with some success.

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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 Science, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, USA

    Hakan Demirci MD

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  1. Hakan Demirci MD
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Correspondence to 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). Orbital and Adnexal Sarcoidosis. 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_4

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  • DOI: https://doi.org/10.1007/978-3-662-46528-8_4

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-46527-1

  • Online ISBN: 978-3-662-46528-8

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