Abstract
Ocular involvement is present in up to 79% of sarcoid patients. Uveitis is the main ocular manifestation and presents as a chronic intraocular inflammatory condition with potentially detrimental effects on visual acuity and quality of life. This retrospective study was conducted to explore the incidence and characteristics of ocular sarcoidosis in a single tertiary ophthalmology center. Medical records of 84 patients presenting between June 2007 and March 2021 were analyzed. Based on the “International Workshop on Ocular Sarcoidosis” (IWOS) criteria, ocular sarcoidosis was determined as: definite (n = 24; 28.6%), presumed (n = 33; 39.3%), probable (n = 10; 11.9%), and indefinite (n = 17; 20.2%) in our study population. In 43.9% of the definite and presumed cases, the eye was primarily affected. In addition to specific ocular findings, the diagnosis was supported by biopsy (28.6%) and chest x-ray or computer tomography (66.7%). Moreover, an increased soluble interleukin-2 receptor (sIL-2R) expression (76.2%), elevated angiotensin-converting enzyme (ACE) levels (34.8%), and lymphocytopenia (35.1%) were valuable laboratory findings. Co-affected organs were lungs (60.7%), skin (15.5%), and central nervous system (8.3%). Our findings support the prominent role of the eye in the early detection of sarcoidosis. In addition to the IWOS criteria, sIL-2R, in particular, was shown to be relevant in establishing the diagnosis.
Highlights
Sarcoidosis is a systemic autoimmune disorder based on the presence of non-caseating, epithelioid cell granulomas after infection or when any other cause has been ruled out
Our 84 patients were clustered into four groups according to the International Workshop on Ocular Sarcoidosis” (IWOS) criteria for the diagnosis of ocular sarcoidosis: definite (n = 24; 28.6%), presumed (n = 33; 39.3%), probable
We investigated how the standard IWOS criteria can be applied and extended to allow for a more reliable and earlier diagnosis
Summary
Sarcoidosis is a systemic autoimmune disorder based on the presence of non-caseating, epithelioid cell granulomas after infection or when any other cause has been ruled out. The clinical course, organ involvement, and prognosis vary considerably and may explain the delay in diagnosis. The disease most frequently involves the lungs in the form of bilateral hilar lymphadenopathy (BHL) or pulmonary infiltration [1]. The prevalence of ocular involvement in systemic sarcoidosis has been reported in a range between 6% to 79%, depending on the country with the highest burden of disease in Japan, followed by Northern Europe and the United States [2,3]. In Europe, a north-south gradient can be observed whereby prevalence is twice as high in the North compared to the South. Middle Eastern countries and China exhibit the lowest burden of disease [2]
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