Results
Demographic
We analysed 56 patients with 101 affected eyes. Of these 56 patients, 25 (44.6%) were male and 31 (55.4%) were female. Mean age at onset of presentation was 31.9 years (range 3–70). The mean age at first visit was 32.2 years and the mean duration of follow-up was just over 60 months. Specific diagnoses included pars planitis 17 (30.4% of entire group), Behcet's disease 9 (16.1%), birdshot choroidopathy 8 (14.3%), primary retinal vasculitis: 7 (12.5%), sarcoidosis 4 (7.1%), Vogt–Koyanagi–Harada syndrome 4 (7.1%), juvenile idiopathic arthritis 1 (1.8%), idiopathic uveitis 3 (5.4%), multiple sclerosis 1 (1.8%), psoriatic arthritis 1 (1.8%), and ulcerative colitis 1 (1.8%). Nine patients (16.1%) were smokers, 36 (64.3%) were non-smokers, and the remaining 11 (19.6%) patients had no recorded information about their smoking history. History of alcohol use was positive in 8 patients (14.3%), negative in 40 (71.4%), and the remaining 8 (14.3%) had no recorded information about their history of alcohol use. Race distribution in our study was mostly non-Hispanic Caucasian (40, 71.4%) followed by Hispanic (6, 10.7%), Asian (4, 7.1%), African–American (2, 3.6%), multicultural (2, 3.6%) and unknown (2, 3.6%). Overall, there were no statistically significant differences found with respect to age, sex, race, or alcohol use with regard to an impact on the course of retinal vasculitis or the response to different lines of treatment.
Clinical Presentation
Retinal vasculitis presented with vascular sheathing, cotton wool spots, and intraretinal haemorrhage. Vascular sheathing occurred without cotton wool spots or intraretinal haemorrhage in 86 eyes (85.2%); vascular sheathing and intraretinal haemorrhage were found in 11 eyes (10.9%); vascular sheathing and cotton wool spots were found in 4 eyes (3.9%); ( Table 1 ). Vasculitis occurred only with arterial involvement in 2 eyes (2.0%), venous involvement occurred in 44 (43.6%), mixed involvement occurred in 13 eyes (12.9%), and the remaining 42 eyes (41.6%) were not specified according to the chart review. Vascular occlusion, defined as complete or near-complete blockage of a vessel as was documented with fluorescein angiography, occurred in 6 eyes (5.9%) in comparison with 95 (94.1%) eyes without occlusion. Retinal vasculitis occurred bilaterally in 45 patients (80.4%) and unilaterally in 11 (19.6%) patients (a total of 101 eyes).
Using the simple linear regression model, Log Mar changes from the baseline visit to the final visit, and from the baseline visit to one-year follow-up were evaluated. Log Mar changes were not related to the following factors: age, sex, race, age of onset, laterality, clinical presentation, obtaining of fluorescein angiography, occlusive vasculitis, and the possible use of an immunomodulator, except for smoking, which was significantly associated with worsening vision, based on Log Mar changes from baseline to final visit (p<0.01) ( Table 2 ). By contrast, we could not show a statistically significant effect for any of these variables, including smoking, for an evaluation based on acuity at 1 year.
We studied the correlation between the decision to use a steroid sparing agent (SSA) and different factors, such as age, sex, race, age of vasculitis onset, time between onset of disease and the time of the baseline evaluation, laterality, clinical presentation, alcohol use and smoking. All these had an insignificant correlation except for age. Age at onset of presentation below 40 years is associated with use of therapy beyond steroids. (OR 3.74, 95% CI 1.29 to 12.52, p=0.01) ( Table 3 ). Also, we assessed the correlation between the presence of ocular complications and other variables such as smoking, alcohol, race, sex, use of fluorescein angiography, occlusive vasculitis and we found that the presence of ocular complications was significantly associated with the use of SSA (OR 4.59, 95% CI 1.78 to 12.34, p<0.01) ( Table 4 ).
Neovascularisation occurred in 7 eyes (7.4%) in the non-occlusive group compared to 6 eyes (100%) in the occlusive group (p<0.01 for group difference, the Fisher's exact test). ERM, noted in 14 eyes ( Table 5 ), or 16.3% of the group which presented with vascular sheathing compared to 9 eyes (60.0%) of the group that presented with sheathing, cotton wool spots and the intraretinal haemorrhage (p<0.01 for group difference, the Fisher's exact test) ( Table 1 ).
Thirty-eight of 56 patients (67.9%) were treated with systemic corticosteroid (oral prednisone), with an average dose over time of 27 mg (range 100–20) and average duration of 13.9 months (range 1–78), 28 (27.7%) eyes were treated with periocular triamcinolone injections, and 4 (4.0%) eyes were treated with intravitreal triamcinolone injections.
Patients with retinal vasculitis were treated with different immunomodulator agents or SSAs, such as methotrexate (MTX) (n=22), mycophenolate (n=12), infliximab (n=8), cyclosporine (n=7), azathioprine (n=7), tacrolimus (n=2), adalimumab (n=2), cyclophosphamide (n=1), sulfasalazine (n=1). mycophenolate+adalimumab (n=1), mycophenolate+cyclosporine (n=2), cyclosporine+MTX (n=2), infliximab+MTX (n=2).