Reconstitution of disrupted photoreceptor layer in uveitis associated with Behçet’s disease by infliximab treatment

Masaru Takeuchi, Kozo Harimoto, Manzo Taguchi, Yutaka Sakurai

Behçet's disease (BD)-associated uveitis causes retinal damage leading to severe visual disturbance. The early morphological changes in the retina are revealed by disappearance or disruption of the external limiting membrane (ELM), inner segment ellipsoid zone (EZ) and cone interdigitation zone (CIZ) in the outer retina shown on spectral domain-optical coherence tomography (SD-OCT). However, it is unknown whether these changes in the retina are reversible in BD-associated uveitis. A 38-year-old man was referred to our hospital with 5 years history of panuveitis in both eyes. Recurrent oral ulcer, folliculitis, and genital ulcer were noted as systemic complications. Moderate cell infiltration into the anterior chamber, and diffuse vitritis were observed in both eyes, and best corrective visual acuity (BCVA) was 20/60 in the right and 20/200 in the left eye. Fluorescein angiography (FA) showed severe dye leakage from extensive retinal vessels in both eyes. Spectral domain-optical coherence tomography (SD-OCT) revealed retinal cysts and disruption of the external limiting membrane (ELM), inner segment ellipsoid zone (EZ) and cone interdigitation zone (CIZ) in the macular region of both eyes. BD was diagnosed based on the ocular features and systemic lesions, and infliximab therapy was initiated for the severe visual disturbance. After treatment with infliximab, foveal excavation was first recovered with disappearance of retinal cysts, and then ELM and EZ were gradually reconstituted on SD-OCT. Finally, CIZ became distinguishable after 24 months of infliximab therapy. BCVA was recovered to 20/25 in both eyes, and ocular inflammatory attack did not recur after the initiation of infliximab therapy. Disruption of ELM, EZ, and CIZ shown on SD-OCT in BD-associated uveitis could be reconstituted by continuous infliximab treatment, which leaded to the improvement of visual acuity.