Ocular Sarcoidosis
Sarcoidosis is a multisystem granulomatous disease that can involve any organ in the body, however it most commonly affects the lungs, lymphatic system, skin, and eye. The diagnostic histopathologic hallmark of sarcoidosis is the presence of non-caseating
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Ocular Sarcoidosis David Ehmann and Sunir Garg
Abstract
Sarcoidosis is a multisystem granulomatous disease that can involve any organ in the body, however it most commonly affects the lungs, lymphatic system, skin, and eye. The diagnostic histopathologic hallmark of sarcoidosis is the presence of non-caseating epithelioid granulomas. Although the etiology of sarcoidosis is unknown, potential infectious sources such as mycobacteria and propionibacteria have been proposed. Ocular sarcoidosis occurs in up to 83% of patients with systemic sarcoidosis and uveitis is the most common ocular manifestation occurring in 50% of patients with ocular sarcoidosis. Both gender and ethnic variance is seen in ocular sarcoidosis with females affected more than males and African Americans more commonly affected than Caucasians. Although 70% of ocular sarcoid presents before age 40, a bimodal distribution in patients aged 20–40 years and 50–60 years occurs. In addition, African American patients generally have an earlier disease onset than Caucasian patients. Patients typically present with symptoms of pain, photophobia, blurred vision, and floaters. After excluding infectious etiologies, the diagnosis of ocular sarcoidosis can be made following the IWOS guidelines, which consist of a combination of ocular signs, blood tests, and imaging modalities. The most common presenting sign of anterior uveitis includes anterior chamber cell and flare with granulomatous (“mutton fat”) keratic precipitates, however 15% present with non-granulomatous keratic precipitates. Early treatment with corticosteroids alone or in combination with immunomodulatory agents can restore vision and prevent blinding complications. As a significant proportion of patients develop chronic disease, regular follow-up with both ophthalmology and internal medicine is required. D. Ehmann • S. Garg (*) Wills Eye Hospital, Philadelphia, PA, USA e-mail: [email protected]; [email protected]; [email protected] © Springer Nature Singapore Pte Ltd. 2018 J. Chhablani et al. (eds.), Retinal and Choroidal Imaging in Systemic Diseases, https://doi.org/10.1007/978-981-10-5461-7_7
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D. Ehmann and S. Garg
Introduction
Sarcoidosis is a multisystem granulomatous disease of unknown origin. It can involve any organ in the body, however it most commonly affects the lungs, lymphatic system, skin, and eye [1–4]. The diagnostic histopathologic hallmark of sarcoidosis is the presence of non-caseating epithelioid granulomas, a key distinguishing factor from other granulomatous diseases such as tuberculosis (Fig. 7.1). Ocular sarcoidosis has been recognized since the early 1900s [5] and its prevalence among patients with known systemic sarcoidosis has been reported to range from 13–83% [1–4]. Although sarcoidosis can involve any part of the eye and surrounding tissue, uveitis is the most common manifestation and occurs in up to 50% of patients [4]. Because 30% of patients with sarcoidosis initially present with uveitis [3] it is important both to be aw
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