Purpose of reviewThe primary systemic vasculitides represent a spectrum of rare but life-threatening conditions that may also affect the eye in various forms. This article reviews recently published data on ocular manifestations of systemic vasculitis.Recent findingsEarly diagnosis and timely treatment has led to better visual outcomes in giant cell arteritis (GCA). Gene expression from orbital tissues could distinguish granulomatosis with polyangiitis (GPA) from sarcoidosis and Graves disease, but not from idiopathic orbital inflammation. Rituximab was an effective therapeutic option in ocular GPA. An ocular attack severity scoring system, flare levels, fluorescein angiography and optical coherence tomography parameters have been suggested as predictors of visual prognosis in Behcet disease. Efficacy of tumor necrosis factor-alpha inhibitors, interferon and anti-interleukin-1 agents was shown in noncontrolled studies of ocular Behcet disease.SummaryWidely used fast-track pathway care as well as ocular imaging to detect subclinical involvement may enable earlier diagnosis of GCA and prevention of permanent visual loss. Orbital inflammation may not remain idiopathic with advances in gene expression profiling of orbital tissues. With an increased availability and the use of biologic agents, visual prognosis will improve in patients with severe ocular complications of systemic vasculitides.