Table 2.
Summary of ocular features reported in VEXAS syndrome in the current literature
| Author | Number of cases | Demographics | Ocular features | |
|---|---|---|---|---|
| Sex | Age (years) (median and range) | |||
| Georgin-Lavialle S et al. [5] | 116 | 111 male (96%) | 67 |
Uveitis = 11 (9.5%) Scleritis = 10 (8.6%) Episcleritis = 14 (12.1%) Orbital mass = 4 (3.4%) |
| Barba T et al. [6] | 1 | Female | 51 | Episcleritis |
| Staels F. et al. [7] | 2 | 2 male (100%) | 69, 75 | Episcleritis = 2 (100%) |
| van der Made CI et al. [8] | 12 | 12 male (100%) | 67 (47–79) |
Posterior scleritis = 1 Blepharitis = 2 Anterior uveitis = 2 Exophthalmos caused by periorbital and infraorbital panniculitis =1 |
| Ciferska H. et al. [9] | 3 | 3 male (100%) | 74 (68–76) |
Sclerouveitis = 1 Episcleritis = 1 |
| Khitri MY et al. [10] | 55 | 53 male (96%) | 66 |
Uveitis = 9 (17%) Scleritis = 7 (13%) Episcleritis = 15 (28%) Retinal vasculitis = 2 (4%) |
| Neupane K et al. [11] | 1 | Male | Early 60s |
Scleritis Choroidal effusion Serous retinal detachment Intraretinal haemorrhage |
| Topilow JS et al. [12] | 1 | Male | 57 | Orbital myositis (medial rectus) |
| Holmes A et al. [13] | 1 | Male | 70 |
Uveitis Periorbital oedema |
| Templé M et al. [14] | 2 | 2 male (100%) | 74, 71 | Retinal vasculitis = 1 (50%) |
| Al-Hakim A et al. [15] | 4 | 4 male (100%) | 56 (49–64) | Periorbital oedema = 1 (25%) |
| Ciprian G [16] | 1 | Male | 56 |
Orbital inflammation Scleritis Orbital myositis (superior rectus) |
| Lacombe V et al. [17] | 6 | 6 male (100%) | 74 (70–78) |
Scleritis or episcleritis = 2 (33.3%) Periorbital oedema = 2 (33.3%) |
| Magnol M et al. [18] | 1 | Male | 57 | Anterior uveitis |
| Kunishita Y et al. [19] | 3 | 3 male (100%) | 69 (66–73) | Scleritis = 2 (66.7%) |
| Lee SMS et al. [20] | 1 | Male | 69 |
Pseudomembranous conjunctivitis Anterior scleritis |
| Lötscher F et al. [21] | 1 | Male | 68 | Scleritis |
| Goyal A et al. [22] | 1 | Male | 64 |
Orbital myositis (medial rectus) Periorbital oedema |
| Midtvedt Ø et al. [4] | 1 | Male | Late 60s | Iridocyclitis |
| Beecher M et al. [23] | 1 | Male | 68 |
Upper lid swelling and oedema Enlarged lacrimal glands |
| Martín-Nares E et al. [24] | 1 | Male | 77 |
Orbital and periorbital inflammation Orbital myositis (medial and inferior recti) Epiphora Eye pain Chemosis |