Ocular Sarcoidosis

Abstract

Ocular disease occurs in approximately a third of sarcoidosis patients. The rate of disease varies around the world, with Japanese sarcoidosis patients having ocular disease in more than 70% of cases. If untreated, ocular disease can lead to permanent visual impairment, including blindness. The most common manifestation is uveitis, with anterior involvement often being self-limiting, whereas posterior involvement can be chronic. The diagnosis of ocular sarcoidosis in patients with known sarcoidosis usually requires a specific examination by an ophthalmologist. For patients presenting with uveitis of unknown etiology, criteria have been proposed for diagnosing ocular sarcoidosis. The treatment of ocular disease ranges from topical therapy to systemic treatments such as methotrexate. Recent reports have demonstrated that monoclonal antibodies blocking tumor necrosis factor can be quite effective for chronic refractory ocular sarcoidosis.

HLA-DRB1* alleles and symptoms associated with Heerfordt's syndrome in sarcoidosis

“…The incidence of eye involvement in patients with sarcoidosis varies from 5% in Finland to .70% in Japan, supporting the notion of a genetic influence on signs of disease presentation [10][11][12]. Other examples of eye involvement are lacrimal gland inflammation, optic neuritis and conjunctival granulomas.…”

“…Anterior uveitis is reported to be more common in AfricanAmericans (70-75%) while posterior uveitis is more common in Caucasians (65-83%) [13]. In patients with sarcoidosis with anterior uveitis, treatment with topical corticosteroids is commonly sufficient, while posterior uveitis usually requires more frequent monitoring and is more difficult to treat [12].…”

Sarcoid Uveitis

“…1,2 The prevalence of ocular involvement observed in clinic-based cohorts has varied widely, from less than 10% in Finland to over 70% in Japan. 3,4 Baughman et al studied 736 patients with biopsy-proven sarcoidosis seen within 10 months from diagnosis at 10 referral centers in the United States and identified eye involvement in 11.8% of patients. 5 While a wide range of ocular manifestations of sarcoidosis have been reported, uveitis appears to be most common, with over 80% of patients with sarcoid uveitis having bilateral disease.…”

“…5 While a wide range of ocular manifestations of sarcoidosis have been reported, uveitis appears to be most common, with over 80% of patients with sarcoid uveitis having bilateral disease. [6][7][8][9][10][11][12][13][14][15][16] Definitive diagnosis of ocular sarcoidosis requires characteristic biopsy findings along with exclusion of other possible causes of ocular inflammation, particularly tuberculosis. 17,18 An International Workshop on Ocular Sarcoidosis (IWOS) met in Tokyo in October, 2006, and through consensus proposed that patients with compatible uveitis and bilateral hilar adenopathy, but no biopsy, be considered presumed ocular sarcoidosis; those with neither a biopsy nor hilar adenopathy, but at least three suggestive intraocular signs and two supportive investigational tests, be considered probable ocular sarcoidosis; and those with a negative biopsy, but at least four suggestive intraocular signs and two supportive studies be classified as possible ocular sarcoidosis.…”

“…Previous cross-cohort comparisons have suggested the possibility of differences in severity and location of sarcoid uveitis based on race and age. [6][7][8][9][10][11][12][13][14][15][16] Gü ngö r et al used enhanced depth imaging (EDI) optical coherence tomography (OCT) to evaluate the choroidal thickness at and within 3000 microns of the fovea in 18 eyes of nine patients with quiescent, biopsy-proven ocular sarcoidosis seen in a uveitis referral clinic in Ankara, Turkey. 21 One patient with quiescent ocular sarcoidosis was receiving low-dose systemic methylprednisolone to control pulmonary disease at the time of the study.…”