Rituximab in refractory Vogt-Koyanagi-Harada disease

Abstract

Introduction: Vogt-Koyanagi-Harada (VKH) prognosis depends on early recognition and treatment; chronic disease may be developed when either delayed or inadequate treatment is performed, whereas other cases despite correct treatment are refractory to different drugs and also become chronic. We report a case of refractory VKH controlled with rituximab treatment.

Case report: A 41-year-old female with painful visual loss and headache was examined. (VA 0.4 in RE and hand movements (HM) in LE). Retinal examination demonstrated multiple serous retinal detachments in both eyes. High-dose oral steroids were started, followed by progressive tapering of prednisone. New acute anterior and posterior relapses were achieved, and other immunommodulators were progressively added-new high-dose steroid treatment, adalimumab, cyclosporine, and methotrexate-but patient had new anterior and posterior recurrences associated with tinnitus and headache. Thus, an infusion of 1 g of rituximab was administered after 15 months follow-up; the VA was 0.2 in RE and counting fingers in LE. Three additional doses of 1 g each were administered 1, 6, and 16 months later. We have achieved a final VA after 34 months follow-up of 0.2 in RE and HM in LE, with definitive control of inflammation, without acute relapses since rituximab was administered.

Conclusion: After searching PubMed/Medline, this is the first report of VKH disease treated with rituximab. Additional studies are warranted to confirm the efficacy of this new approach for inflammatory control in refractory cases of VKH disease.

Fig. 1

a, b. Color fundus images of right and left eye showed disc edema and multifocal serous retinal detachment with foveal involvement. c, d. Fluorescein angiography photographs of both eyes revealed characteristic pinpoint hyperfluorescence in intermediate time. e, f. Time domain optical coherence tomography cross-sectional horizontal image showed typical multilobulated retinal serous detachment in both eyes

Fig. 2

Optical coherence tomography cross-sectional horizontal images of right and left eye (RE and LE, respectively); a (2 days follow-up) showed response to high-dose corticosteroids in both eyes with decrease of neuroretinal detachment that is more evident after 2 weeks of follow-up in b. c (3 months follow-up) revealed first recurrence of macular detachment in left eye first and later in right eye (d, 4 months follow-up) treated with a new high-dose oral corticosteroid therapy associated with bilateral intravitreal injection of ranibizumab and dexamethasone and adalimumab every 15 days. f (5 months follow-up) showed reappearance of retinal detachment, so we added treatment with cyclosporine (100 mg per day) and methotrexate (17.5 mg weekly). g (7 months follow-up) revealed macular detachment, and examination showed recurrence of anterior and posterior inflammation with tinnitus and headache; thus, we started a first infusion of 1 g of rituximab (15 months after onset of symptoms)

Fig. 3

a, b. Color fundus photography of right and left eye, respectively, after 30 months follow-up showed typical diffuse athrophy of retinal pigment epithelium leading to a characteristic image of sunset-glow fundus. a (arrows) revealed the presence of Dalen-Fuchs nodules