Treatment and patient characteristics
Patients received regimens of corticosteroid pulse therapy according to the timing of their first visit. In the 27 patients administered intravenous methylprednisolone, the dose was 1,000 mg/day for 3 consecutive days followed by tapering of oral prednisolone (i.e., pulse therapy), as described previously . Oral prednisolone was temporarily increased or restarted in the event of anterior or posterior recurrence of VKH disease. The remaining two patients were administered intravenous prednisolone and tapered from 100 mg/day (i.e., high-dose therapy), as described previously . Oral cyclosporine was not administered in the present study. We calculated duration from onset to steroid administration and duration from first visit to steroid administration in all patients. Mean duration from onset to steroid administration was 17.4±10.7 days and mean duration from first visit to steroid administration was 4.1±3.2 days. Baseline patient characteristics are summarized in Table 1. The mean follow-up was 22±20 months (range 6–105 months). Recurrences of inflammation were observed in 5 patients (17%) over follow-up, and they consisted of visible posterior segment inflammation (subretinal fluid or choroidal white lesions) in all patients.
In visual acuity, mean logMAR best-corrected visual acuity (BCVA) values at baseline, at 3 months after treatment and, at 6 months after treatment were 0.34±0.58, 0.01±0.25,and 0.01±0.38 in HLA-DRB1*04 -/-, -0.16±0.33, -0.11±0.06,and -0.06±0.14 in HLA-DRB1*04 +/-, and 0.008±0.14, -0.13±0.05, and -0.09±0.24 in HLA-DRB1*04 +/+, indicating significant visual improvements from baseline after treatment in HLA-DRB1*04 +/- and HLA-DRB1*04 +/+group ( 3M : p<0.01, p<0.01, p=0.056, respectively) ( 6M:p=,0.24, p<0.01, p=0.07,respectively). In covariate analysis considering the influence of baseline visual acuity, the HLA-DRB1*04 -/- and HLA-DRB1*04 +/- groups showed significant differences in the final visit (p<0.01). Mean CCT values pretreatment and at final visit after treatment were 489.4±258.4 and 285.2±159.4 μm in HLA-DRB1*04 -/-, 557.5±197.5 and 356.1±135.5 μm in HLA-DRB1*04 +/-, and 517.5±190.5 and 375.8±211.2 μm in HLA-DRB1*04 +/+, indicating changes compared with baseline (p=0.110, p<0.01, p=0.173, respectively). In the HLA-DRB1*04 +/- group alone, mean post-treatment CCT values were significantly decreased compared with baseline CCT.
Correlation with HLA-DRB1*04 allele
HLA-DRB1*04 typing was performed for 5 patients (10 eyes) in normal subjects (HLA-DRB1*04 -/-), 15 patients (29 eyes) in heterozygotes (HLA-DRB1*04 +/-), and 6 patients (12 eyes) in homozygotes (HLA-DRB1*04 +/+). Linear regression analysis showed significant differences among the three groups of homozygotes, heterozygotes, and normal subjects in logMAR BCVA at baseline in Figure 1 (p<0.01).
Comparisons of clinical parameters at post-treatment in the three groups are summarized in Table 2. Linear regression analysis found significant differences among the three groups of homozygotes, heterozygotes, and normal subjects in logMAR BCVA at three months after treatment in Figure 2 (p<0.01). There was no significant differences at six months after treatment (p=0.25). This result was consistent with the finding of no difference among three groups with regard to choice of treatment regimen according to the period during which the patient visited the clinic (i.e., large dose, earlier; pulse, later). Second, the number of recurrences during follow-up did not differ between the three groups.
In terms of visual prognosis of the 57 eyes examined, we documented mild to moderate cataracts in 4 eyes and drug-controllable glaucoma in 3 eyes after treatment, none of which required surgery during follow-up. No differences in the frequency of these ocular complications were seen among patients.