Table 2 Summary of ocular features reported in VEXAS syndrome in the current literature

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