Patients with retinal vasculitis rarely suffer from systemic vasculitis

Abstract

Objectives: Systemic vasculitis is often mistakenly assumed to be a common cause of retinal vasculitis. We sought to determine the relationship between retinal vasculitis and systemic vasculitis.

Methods: A selected review was performed on 1390 charts of patients attending the uveitis clinic at the Oregon Health and Science University between 1985 and 2010. Included in the review were all patients with diagnoses commonly associated with retinal vasculitis and all patients who were diagnosed with a systemic vasculitis. Retinal vasculitis was identified by perivascular exudates, intraretinal hemorrhage, or cotton wool spots as seen on clinical examination or by vascular occlusion or leakage as identified by fluorescein angiogram.

Results: Two hundred seven or 14.9% of patients with uveitis had retinal vasculitis as a component of the intraocular inflammation. Thirty-five patients had retinal vasculitis that was primary, ie, not associated with a systemic disease, and the dominant manifestation of the uveitis. Fourteen of the patients with retinal vasculitis had Behcet's disease. Only 11 of the 1390 patients with uveitis had a systemic vasculitis. Of these 11, four had retinal vasculitis including 1 secondary to a cytomegalovirus retinitis. Thus, systemic vasculitis was directly responsible for 1.4% or 3 of 207 cases of retinal vasculitis. Nonvasculitic systemic diseases such as sarcoidosis (n = 13), syndromes confined to the eye such as pars planitis (n = 36), and intraocular infections (n = 29) were far more common causes of retinal vasculitis.

Conclusions: Retinal vasculitis is a relatively common feature of uveitis. Patients with retinal vasculitis, however, rarely suffer from 1 of the classical systemic vasculitides.

Figure 1

Retinal photograph from a patient with primary retinal vasculitis. Photograph illustrates cotton wool spot, vascular sheathing, and intraretinal hemorrhage.

Figure 2

Retinal photograph from a patient with eosinophilic granulomatosis with polyangiitis. An area of white retinitis is surrounded by a pigmented scar. An occluded vessel affected by vasculitis runs through the center of the lesion. The retinitis developed while the patient was receiving immunosuppression. It was attributed to CMV infection and it responded to intravitreally injected antiviral therapy and a reduction in the immunosuppression.