Patients with Retinal Vasculitis Rarely Suffer from Systemic Vasculitis
Department of Ophthalmology, Oregon Health and Science University, Portland, OR 97239, USA. Seminars in arthritis and rheumatism
(Impact Factor: 3.93).
12/2011; 41(6):859-65. DOI: 10.1016/j.semarthrit.2011.10.006
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.
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.
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.
Retinal vasculitis is a relatively common feature of uveitis. Patients with retinal vasculitis, however, rarely suffer from 1 of the classical systemic vasculitides.
Available from: PubMed Central
- "The contribution of BD on the overall incidence of retinal vasculitis can vary based on the population at risk. A review of 1390 uveitis cases on the west coast of the United States found 207 patients with evidence of retinal vasculitis; of these cases, only 14 patients had BD . On the other hand, retinal vasculitis is common among patients with ocular BD. "
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ABSTRACT: Ischemic retinal vasculitis is an inflammation of retinal blood vessels associated with vascular occlusion and subsequent retinal hypoperfusion. It can cause visual loss secondary to macular ischemia, macular edema, and neovascularization leading to vitreous hemorrhage, fibrovascular proliferation, and tractional retinal detachment. Ischemic retinal vasculitis can be idiopathic or secondary to systemic disease such as in Behçet's disease, sarcoidosis, tuberculosis, multiple sclerosis, and systemic lupus erythematosus. Corticosteroids with or without immunosuppressive medication are the mainstay treatment in retinal vasculitis together with laser photocoagulation of retinal ischemic areas. Intravitreal injections of bevacizumab are used to treat neovascularization secondary to systemic lupus erythematosus but should be timed with retinal laser photocoagulation to prevent further progression of retinal ischemia. Antitumor necrosis factor agents have shown promising results in controlling refractory retinal vasculitis excluding multiple sclerosis. Interferon has been useful to control inflammation and induce neovascular regression in retinal vasculitis secondary to Behçet's disease and multiple sclerosis. The long term effect of these management strategies in preventing the progression of retinal ischemia and preserving vision is not well understood and needs to be further studied.
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To examine the characteristics and visual outcome in 207 patients with retinal vasculitis.
Demographic and visual outcome data were collected retrospectively from the ophthalmologic records of 207 cases (321 affected eyes). Descriptive analysis was performed on all cases and visual outcome analysis was performed for the 114 cases with visual acuity recorded at 2 or more visits. The Kaplan-Meier method and Cox regression were used to examine visual outcome and predictors for prognosis.
Patients in our series had a roughly even distribution of sex, were predominantly non-Hispanic white (77.8%), and had bilateral disease (75.7%). The annualized mean visual acuity change for the 203 eyes (114 patients) with some follow-up was 0.01 logMAR unit per year. Although 75 eyes (36.9%) had 20/25 or better visual acuity at baseline, 33.6% of the remaining eyes experienced visual acuity improvement of at least 2 lines on the Snellen chart during follow-up and some continued to improve more than 9 years after the initial evaluation. Cox multivariate analysis demonstrated that patients who were nonwhite, had worse visual acuity at baseline, or who had an ocular infection were more likely to experience improvement by this definition.
We believe that this is the first US case series to investigate visual outcome in patients with this diagnosis. Although many patients in our series worsened despite therapy, a subset experienced substantial improvement.
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To report on the clinical features and etiology of patients with retinal vasculitis (RV).
Materials and Methods:
We reviewed medical records of 47 patients (75 affected eyes) diagnosed with RV. Clinical presentations, ocular complications, associated systemic diseases, and treatment regimens were registered.
Etiology of RV included infectious causes in 10/47, (21%) while an association with systemic and/or ocular non-infectious disorders was noted in 22/47 (47%). Eales’ disease and Behcet's disease represented the most common clinical entities in non-infectious group while tuberculosis-associated RV was diagnosed in 6/10 (60%) among those with infectious disorders. RV was bilateral in 28/47 (60%) patients. Retinal veins were most commonly affected (72%, 34/47). Involvement of arteries was present in 12/47 (25%) and was associated with viral infections and Behcet's disease. Ocular complications developed in 60/75 (80%) eyes. The most common complications were elevated intraocular pressure and/or glaucoma (33/75, 44%). Retinal detachment, vitreous hemorrhage, and cystoid macular edema developed in similar percentages (15%).
RV in Thailand manifested mostly in male patients, was typically bilateral and involved mostly veins. Involvement of arteries was observed in patients with viral infections and Behcet's disease. Tuberculosis was the most common infectious cause.
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