Table 3.
Characteristics and common features of sarcoid uveitis in adults, children and Blau Syndrome/ Early Onset Sarcoidosis (BS/EOS)
| Study | nbr of patients in the study with ocular manifestation of sarcoidosis or BS/EOS | Number of all patients in the study | Age, years, median of patients’ age with ocular manifestations of sarcoidosis or BS/EOS; for BS/EOS median age at ocular onset and age at study baseline | Bilateral | Granulomatous | Mutton-fat keratic precipitates | Mutton-fat KPs, iris nodules, or both | Non granulomatous | |
|---|---|---|---|---|---|---|---|---|---|
| Adults | SUN, 2021 | 278b | 2684 | 49 (IQR 39–61) | 82% | - | 23% | 35% | - |
| Acharya,2018 | 167c | 884 | 49 (IQR 39—60) | 86% | - | 35% | 46% | - | |
| Coulon, 2019 | 194d | 194 | 52.1 ± 17.8 | 77.8% | 60% | - | - | - | |
| Niederer, 2021 | 362e | 362 | 46 (IQR 35–57) | 87% | 48% | - | - | - | |
| Allegri, 2022 | 235 | 235 | mean age 52 | 85% | 52% | - | - | - | |
| Children > 5 years | Choi, 2011 | 13f | 460 | 12 (range: 5 to 16) | - | 31% | - | - | 62% |
| Morelle, 2019 | 9b | 147 | -, mean age 10.5 (range: 7–14) | 100% | 23% | - | - | - | |
| Waduthantri S, 2021 | 8 g | 73 | mean age 12 (range: 5–15) | - | - | - | - | - | |
| Blau Syndrome / Early Onset Sarcoidosis | Sarens, 2018 | 38 | 50 | 5 (range 0,5- 48); at study baseline: 17 (range 2–56) | 97% | - | - | - | - |
| Kumrah, 2022 | 9 | 11 | 4 (range: 2–26); median age at dignosis: 9 (range: 2–26) | 100% | 44% | - | 11% only iris nodules specified | 11% | |
| Matsuda, 2020 | 38 | 50 | -, -; all patients in the study: mean 26.7 (range 0–61) | - | - | - | - | - | |
| Wu,2019 | 7 | 7 | 5,5 (range 2-24y), at study baseline: 10,5 (range: 4–34) | - | - | - | - | - | |
| Babu, 2020 | 7 | 7 | -, at study baseline: 9 (range 2,5–25) | 71%i | 71%j | 14% | - | - | |
| Rosé, 2015 | 25 | 31 | 4.4 (range 0,5–22); at study baseline 16.5 (range: 1.9–58) | 96% | - | - | - | - |
| No keratic precipitates | Anteriora uveitis | Intermediatea uveitis | Posteriora uveitis | Panuveitis a | Multifocal choroiditis | Retinal vascular inflammationh | Solitary choroidal nodule (optic disc nodules or granulomas) | |
|---|---|---|---|---|---|---|---|---|
| Adults | 52% | 40% | 19% | 4% | 37% | 30% | 18% | 2% (-) |
| - | 20% | 3% | 9% | 67% | 45% | 36% | 3% (4%) | |
| - | 34% | 10% | 7% | 49% | 40% | 30% | - | |
| - | - | - | - | - | 43% | 21% | 11% isolated choroidal or optic nerve granuloma | |
| - | 52% | 10% | 29% | 8% | - | - | - | |
| Children > 5 years | 8% | - | - | - | - | 54% | 31% | - |
| - | 77% | - | - | 23% | - | - | - | |
| - | 12.50% | - | 25% | 62.50% | - | - | - | |
| Blau Syndrome / Early Onset Sarcoidosis | - | 29% | - | - | 51% | 39% | 0% | - (12%) peripapillary nodules |
| - | 33% | - | - | 67% | 67% | 11% | - | |
| - | - | - | - | - | - | - | - | |
| - | 14% | - | - | 85% | - | - | - | |
| - | 29% | - | - | 43% | - | - | 14% (-) | |
| - | - | - | - | - | - | - | - |
IQR interquartile range; data not available
aanatomic class (SUN)/ type by anatomic location (Acharya, 2018)
bsarcoidosis-associated uveitis
cincluding 98 patients with definite ocular sarcoidosis and 69 patients with presumed ocular sarcoidosis according to IWOS 2009 criteria
dincluding 145 with biopsy-proven and 49 with presumed sarcoid uveitis following the WASOG/ATS/ERS criteria
eincluding definite or presumed ocular sarcoidosis according to IWOS 2009 criteria
fincluding 4 definite, 3 presumed, or 6 probable sarcoidosis according to selfestblished scoring system
g8 patients with presumed ocular sarcoidosis and 2 patients with sarcoid scleritis
hrefered to as: retinal vascular sheathing or periphebitis or vasculitis
iat least 71%, no data available on bilaterality for 2 other patients with only conjunctival granulomas
jand additionally 29% with only conjunctival granulomas
- no data available