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 & 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 207 or 14.9% of patients with uveitis had retinal vasculitis as a component of the intraocular inflammation. Thirty-five patients had retinal vasculitis which was primary, i.e. 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 one secondary to a CMV retinitis. Thus, systemic vasculitis was directly responsible for 1.4% or 3 of 207 cases of retinal vasculitis. No-vasculitic 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. Conclusion Retinal vasculitis is a relatively common feature of uveitis. Patients with retinal vasculitis, however, rarely suffer from one of the classical systemic vasculitides.

The course of retinal vasculitis

“…Retinal vasculitis typically occurs in association with intraocular inflammation as in sarcoidosis or as an isolated, idiopathic entity, but surprisingly, it rarely occurs in association with systemic forms of vasculitis 15. Patients with idiopathic retinal vasculitis are typically young adults with no signs or symptoms suggestive of an underlying systemic or ocular disease 17.…”

“…We have recently reported on 207 patients with retinal vasculitis who were evaluated at the Oregon Health & Science University, Casey Eye Institute, Portland, between the years 1985 and 2010 15 16. This report is based on a subset of that group, 56 patients with 101 affected eyes (45 patients were affected bilaterally and 11 patients were affected unilaterally).…”

“…We recently analysed a large cohort of patients with retinal vasculitis who had been seen at the Casey Eye Institute, Oregon Health & Science University 15 16. We found that many patients maintained excellent visual acuity, while others did less well.…”

Retinal vasculitis: a novel paradoxical effect of anti-TNFα?

“…RV can be idiopathic or with greater frequency associated to other ocular or systemic diseases 4. The most common diseases observed are Behçet’s disease, sarcoidosis and multiple sclerosis 5. In 2012, Rosenbaum et al reviewed the charts of 1390 patients with uveitis and found evidence of RV in 207 patients.…”

Uveitis in adults: What do rheumatologists need to know?

“…The less common causes are in italic type [8][9][10][11]. [6]. These lesions may lead to loss of vision.…”