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Orbital and Adnexal Sjögren Syndrome

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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

Sjögren syndrome (SS) is a systemic autoimmune disorder in which dryness of mucosal surfaces results from inflammation of exocrine glands. This may be associated with (secondary, sSS) or without (primary, pSS) connective tissue disorders such as scleroderma, rheumatoid arthritis, or systemic lupus erythematosus. Inflammation of exocrine glands leads to clinical manifestations of dry eye and dry mouth, among other extraglandular manifestations including involvement of the skin, joints, liver, pancreas, thyroid gland, and hematologic, neurologic, and cardiovascular systems. An increased risk of cancer, most commonly non-Hodgkin’s lymphoma, has been observed in patients with pSS, highlighting the need for close monitoring of these patients. Diagnosis of pSS is based on a combination of clinical signs and symptoms, histopathology of biopsy specimens, and laboratory testing. Ocular signs and symptoms are secondary to aqueous tear deficiency from immune cell infiltration and destruction of the lacrimal gland. They include a spectrum that extends from dry eye symptoms to squamous metaplasia, persistent epithelial defects, ulceration, and risk for infection. Management of SS is graded based on disease severity, and treatment of ocular involvement varies from topical lubricants to supplement aqueous production, reduction in tear drainage by occlusion of the lacrimal puncta, use of topical anti-inflammatory medications, and oral secretagogues. Systemic immunosuppressive therapy may improve systemic disease manifestations, but the effect on SS-related dry eyes has yet to be firmly established.

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Compliance with Ethical Requirements

Shivani Gupta and Hakan Demirci declare that they have no conflict of interest. No human studies were carried out by the authors for this article. No animal studies were carried out by the authors for this article.

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Authors and Affiliations

  1. Department of Ophthalmology and Visual Science, W.K. Kellogg Eye Center, University of Michigan, 1000 Wall Street, Ann Arbor, MI, 48105, USA

    Shivani Gupta MD, MPH

  2. Veterans Administration Ann Arbor Health Care System, Ann Arbor, MI, USA

    Shivani Gupta MD, MPH

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

    Hakan Demirci MD

Authors
  1. Shivani Gupta MD, MPH
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  2. Hakan Demirci MD
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Corresponding author

Correspondence to Shivani Gupta MD, MPH .

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Editors and Affiliations

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

    Hakan Demirci

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Gupta, S., Demirci, H. (2015). Orbital and Adnexal Sjögren Syndrome. 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_5

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  • DOI: https://doi.org/10.1007/978-3-662-46528-8_5

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-46527-1

  • Online ISBN: 978-3-662-46528-8

  • eBook Packages: MedicineMedicine (R0)

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