Purpose: Scleritis may occur associated with systemic vasculitic disea
ses. The detection of systemic vasculitic diseases in patients with sc
leritis is a sign of poor general prognosis because it indicates poten
tially lethal systemic complications. This study was undertaken to ana
lyze the ocular prognosis of patients with scleritis and the different
systemic vasculitic diseases. Methods: Patient characteristics, scler
itis type, and ocular complications were evaluated in 82 patients with
scleritis with systemic vasculitic diseases; comparisons were made be
tween patients with scleritis with a specific systemic vasculitic dise
ases and patients with scleritis with the other systemic vasculitic di
seases. Results: Patients with scleritis with Wegener granulomatosis h
ad more necrotizing scleritis (79%, P = 0.0001), decrease in vision (7
9%, P = 0.014), and peripheral ulcerative keratitis (50%, P = 0.0139)
than patients with scleritis with the other systemic vasculitic diseas
es. Patients with scleritis with spondyloarthropathies had less decrea
se in vision (8%, P = 0.001) and peripheral ulcerative keratitis (0%,
P = 0.0256) than patients with scleritis with the other systemic vascu
litic diseases. Patients with scleritis and systemic lupus erythematos
us had less necrotizing scleritis (0%, P = 0.0412) than patients with
scleritis with the other systemic vasculitic diseases. Conclusions: Oc
ular prognosis of scleritis with systemic vasculitic diseases varies d
epending on the specific systemic vasculitic diseases: scleritis in sp
ondyloarthropathies or in systemic lupus erythematosus is usually a be
nign and self-limiting condition, whereas scleritis in Wegener granulo
matosis is a severe disease that can lead to permanent blindness; scle
ritis in rheumatoid arthritis or relapsing polychondritis is a disease
of intermediate severity, which should be monitored closely for the d
evelopment of ocular complications.