BACKGROUND: The Sarcoidosis Diagnostic Score (SDS) system has been established for sarcoidosis patients based on the WASOG organ involvement criteria. We evaluated modifications of the SDS system to determine if they improved its the diagnostic accuracy.
METHODS: Biopsy-confirmed patients with sarcoidosis seen during a 7-month period at 9 sarcoidosis centers across the world. Patients with non-sarcoidosis seen at the same sites were served as control patients. Comparing the SDS-biopsy and SDS-clinical values of five groups: duration of symptoms prior to evaluation (≤1 years vs.>1 years, ≤2 years vs.>2 years), organ involvement (lung, eye, or cardiac), race, and sex.
RESULTS: A total of 990 patients with sarcoidosis and 1011 controls were included in this study. The SDS-clinical was significantly more discriminating for those undergoing assessment with symptoms for more than one year (z-statistic=2.570, p = 0.0102) or two years (z-statistic=2.546, p = 0.0109). However, the addition of two points for both >1 years and >2 years since onset of symptoms did not increase sensitivity and specificity of diagnosis with the SDS system. The SDS-clinical cut-off for patients with ocular or cardiac disease was two points higher than that for lung disease. There was no difference in SDS-clinical or biopsy AUC values based on gender or race.
CONCLUSIONS: The longer the duration of symptoms prior to diagnosis, the more likely the diagnosis of sarcoidosis was correct. For patients presenting with ocular or cardiac symptoms, evidence of multi-organ involved can improve the diagnostic accuracy of the SDS-clinical.

OBJECTIVE: To evaluate possible risk factors for uveitis among Finnish sarcoidosis patients.
METHODS: Patient charts of patients with sarcoidosis, with (n = 97) or without (n = 255) uveitis, and with a comprehensive eye examination from January 2014 to January 2021 at Tays Eye Centre, Tampere University Hospital, Finland were studied.
RESULTS: Sarcoidosis patients with uveitis had higher rate of lymphocytopenia (43% vs. 29%, p = 0.041) and lower serum lysozyme levels (2.0 mg/L vs. 2.3 mg/L, p = 0.049; 95% CI, -0.692 to -0.002). Lysozyme level or lymphocytopenia did not have a statistically significant effect on the probability of uveitis in a binary logistic regression analysis. No other differences in the potential risk factors with p-values ≤0.05 were found, including bilateral hilar lymphadenopathy, serum angiotensin-converting enzyme (ACE) levels, sex, age and history of smoking.
CONCLUSIONS: Lymphocytopenia and lower serum lysozyme levels present as possible risk factors for uveitis among patients with sarcoidosis. Systematic measurement of lymphocyte and lysozyme levels in sarcoidosis is needed to further understand their role as potential risk factors.

UNASSIGNED: Uveitis is a common ocular manifestation in individuals with sarcoidosis, a multisystem inflammatory disorder. This study aimed to explore clinical and genetic factors associated with the presence or absence of uveitis in sarcoidosis patients.
UNASSIGNED: Total 625 Dutch sarcoidosis patients were included. Among these, 170 underwent ophthalmic examination, and 61 were diagnosed with uveitis. Demographic and clinical data, including age, gender, race, biopsy status, chest radiography findings, TNF-α inhibitor treatment, and uveitis classification were collected retrospectively from medical records. Genetic data was available for HLA haplotypes, TNF-α G-308A, and BTNL2 G16071A polymorphisms.
UNASSIGNED: The majority of the patients presented with bilateral uveitis (80.3%). The proportion of women was higher in the uveitis group compared to the non-uveitis group (67.2% and 47.7%; p = 0.014). Pulmonary involvement (chest radiographic stage II-III) was significantly lower in patients with uveitis (36.1% versus 64.2%; p < 0.001). Patients with uveitis were more often treated with TNF-α inhibitors (67.2% versus 29.4%; p < 0.001) and the outcome was better compared with the non-uveitis group, 92% vs 68%, responders (p < 0.012). Uveitis patients treated with TNF-α inhibitors (either adalimumab or infliximab) were more likely to suffer from intermediate or posterior uveitis than anterior uveitis. Genetic analysis identified a significant association between the BTNL2 G16071A GG genotype and uveitis (p = 0.012).
UNASSIGNED: This study highlights distinctive demographic, clinical and genetic features associated with uveitis in sarcoidosis patients. Ocular sarcoidosis was more prevalent in women. Further research is warranted to explore the implications of these findings for treatment strategies and prognostic assessments.

OBJECTIVE: To evaluate and compare characteristics, diagnosis, treatment, visual prognosis, and course between ocular sarcoidosis with or without uveitis in a population in Southern France.
METHODS: We retrospectively analyzed data from patients with ocular sarcoidosis in a tertiary eye care center in Nice from January 2003 to December 2021. The inclusion criterion was biopsy-proven ocular sarcoidosis according to IWOS criteria as the first clinical manifestation of sarcoidosis.
RESULTS: A total of 25 patients were included. Twenty patients had uveitis (70% panuveitis, 20% intermediate uveitis, and 10% anterior uveitis) and five patients had non-uveitic ocular sarcoidosis (one patient with dacryoadenitis, one patient with orbital granuloma, two patients with palpebral granuloma, and one patient with episcleritis). Only the cases with uveitis had bilateral involvement (85% of cases). There was no significant difference in ethnicity, biopsy diagnosis, systemic manifestations, or treatment between the two groups. Final visual outcomes remained good for both groups, with 96% of patients with BCVA>20/50, with no significant difference. Patients with non-uveitic sarcoidosis experienced less recurrence on treatment (P=0.042) and more remission (P=0.038) than patients with uveitis. Eighty percent of patients with uveitis had at least three suggestive clinical intraocular signs meeting IWOS criteria.
CONCLUSIONS: In this population in Southern France, uveitis was the most common presentation of ocular sarcoidosis. The type of ocular sarcoidosis does not appear to be correlated with the type of systemic manifestations, use of systemic therapy, or visual prognosis, but patients with non-uveitic ocular sarcoidosis appear to have a better course with fewer recurrences on treatment and more remission than patients with uveitic ocular sarcoidosis.

Ocular sarcoidosis most commonly presents with symptoms and is first diagnosed before systemic sarcoidosis in at least half of the patients with sarcoidosis. Prevalence of ocular involvement in sarcoidosis varies between 2-80% depending on the study setting, included ocular diseases, and studied population. In many studies, ocular involvement in sarcoidosis has been overestimated mainly because study populations have been collected from eye clinics and because the study criteria have included ocular findings or symptoms that do not require treatment or monitoring. In a screening setting, asymptomatic ocular sarcoidosis has been detected in only 2-5%. 0-1% of the screened sarcoidosis patients have required treatment. For these reasons, ocular screening in sarcoidosis seems generally of little value. Patients with sarcoidosis who present with ocular symptoms should be screened for ocular sarcoidosis in a timely manner because they are at high risk of ocular disease.

Sarcoidosis is a chronic inflammatory granulomatous disease of unknown aetiology. Although the lungs and thoracic lymph nodes are the most frequently affected structures, every tissue can be affected. Ophthalmological involvement occurs in 25 % of cases. We report a clinical case in which the diagnosis of sarcoidosis was revealed by isolated unilateral optic disc edema.
La sarcoïdose est une pathologie inflammatoire granulomateuse chronique dont l’étiologie reste inconnue. Bien que les poumons et les ganglions thoraciques soient les structures les plus fréquemment atteintes, tous les tissus peuvent être affectés. L’atteinte ophtalmologique est présente dans 25 % des cas. Nous rapportons un cas clinique dont le diagnostic de sarcoïdose fut révélé par un œdème papillaire unilatéral isolé.

To evaluate retinal and choroidal alterations in eyes with inactive ocular sarcoidosis and to compare the findings with healthy controls.
This cross-sectional study included 11 patients with inactive ocular sarcoidosis (study group) and 11 healthy individuals (control group). Retinal and choroidal vascular differences were evaluated using optical coherence tomography angiography (OCTA) and enhanced depth imaging-optical coherence tomography (EDI-OCT).
A total of 21 eyes of ocular sarcoidosis patients without active inflammation and 22 eyes of healthy controls were analyzed. The mean whole-image deep capillary plexus vessel density was significantly lower in the study group than in the control group (P = 0.03). The acircularity index values were significantly higher, and the foveal density-300 values were significantly lower in the study group than in the control group (P = 0.01, both). The flow areas of the choriocapillaris at 1-, 2-, and 3-mm radius areas were also significantly lower in the study group (all P < 0.01). The mean choroidal thickness (CT) was significantly lower in all regions between nasal and temporal at 3000 μm in the study group (all P < 0.05). The choroidal vascularity index (CVI) values were also significantly lower in the study group (P < 0.01).
Ocular sarcoidosis was associated with a reduction in CT and the CVI with a decrease in retinal vessel density and choriocapillary flow parameters. The combination of OCTA and EDI-OCT imaging may be useful in monitoring ocular sarcoidosis eyes to detect alterations in the retinal and choroidal layers.

UNASSIGNED: To evaluate the ocular features, course, and prognosis of uveitis in the ocular sarcoidosis (OS) subgroups without previously known systemic sarcoidosis.
UNASSIGNED: Ninety-one eyes of 49 patients were included. Definite OS patients were classified as group 1 (n = 15), presumed OS patients as group 2 (n = 15), and probable OS patients as group 3 (n = 19).
UNASSIGNED:  The most common presentation of OS was panuveitis in all groups. During the follow-up, bilateral ocular involvement was observed in 85.7% of the patients. The most common extraocular involvement was pulmonary involvement, which was detected in 61.2% of the patients at the time of diagnosis.
UNASSIGNED: Ophthalmologists should be aware that uveitis may be the first sign of systemic sarcoidosis. In all subgroups of OS, uveitis tends to be bilateral and the most common type is panuveitis. Therefore, the eye without obvious clinical findings should be carefully evaluated clinically and angiographically.

OBJECTIVE: Systematic ocular screening is recommended in sarcoidosis, because of a high rate of ocular involvement. The purpose of this study was to determine whether ocular screening is useful in sarcoidosis in a Finnish university hospital population with 0.5 M inhabitants.
METHODS: Patient charts of patients with sarcoidosis, without a history of ocular sarcoidosis, without ocular inflammatory symptoms, and with a comprehensive eye exam from January 2014 to January 2021 at Tays Eye Centre, Tampere, Finland, were studied.
RESULTS: Five of 262 patients (2%) were diagnosed with asymptomatic uveitis. No other types of ocular sarcoidosis were found. Anterior uveitis without complications was present in three patients, unilaterally in two and bilaterally in one patient. Posterior uveitis was present in two patients, a unilateral choroidal granuloma requiring treatment in one and bilateral punched-out chorioretinal lesions in the other patient.
CONCLUSIONS: With this low rate of ocular involvement requiring treatment in sarcoidosis, systematic screening for asymptomatic ocular sarcoidosis does not seem useful in a Finnish population. In Tays Eye Centre, systematic screening of ocular sarcoidosis was discontinued in 2021.

Sarcoidosis is an inflammatory disease that involves the eyes in 10-55% of cases, sometimes without systemic involvement. All eye structures can be affected, but uveitis is the most common ocular manifestation and causes vision loss. The typical ophthalmological appearance of these uveitis is granulomatous (in cases with anterior involvement), which are usually bilateral and with synechiae. Posterior involvement includes vitritis, vasculitis and choroidal lesions. Tuberculosis is a classic differential diagnosis to be wary of, especially in people who have spent time in endemic areas. The diagnosis is based on histology with the presence of non-caseating epithelioid granulomas. However, due to the technical difficulty and yield of biopsies, the diagnosis of ocular sarcoidosis is often based on clinico-radiological features. The international criteria for the diagnosis of ocular sarcoidosis have recently been revised. Corticosteroids remain the first-line treatment for sarcoidosis, but up to 30% of patients require high doses, justifying the use of corticosteroid-sparing treatments. In these cases, immunosuppressive treatments such as methotrexate may be introduced. More recent biotherapies such as anti-TNF are also very effective (as they are in other non-infectious uveitis etiologies).