Antiretinal antibody- proven autoimmune retinopathy

Abstract

A young female presented with bilateral subacute onset of progressive decrease in night vision and reduced peripheral field of vision. The short duration and rapid progression of symptoms along with the lack of family history of night blindness prompted a diagnosis of autoimmune retinopathy (AIR). Fundus fluorescein angiography, optical coherence tomography, visual fields, and electroretinogram were suggestive of AIR. A differential diagnosis of retinitis pigmentosa (RP) was also made. Antiretinal autoantibodies were detected in the blood sample. Treatment was with oral steroids and subsequently oral immunosuppressive agents. Visual acuity was maintained, fundus examination reverted to normal, and investigations repeated at every visit were stable with improvement in visual fields. Our case suggests that AIR, if diagnosed early and treated appropriately, may have a good outcome and should be considered in patients with an atypical presentation of RP.

Figure 1

Color fundus photograph showing normal discs, attenuated vessels, retinal pigment epithelial mottling, and suspicion of cystoid macular edema

Figure 2

Fundus fluorescein angiogram showing perivenous patchy staining, retinal pigment epithelial defects, and disc staining

Figure 3

Normal fundus autofluorescence

Figure 4

Optical coherence tomography showing cystic spaces

Figure 5

Electroretinogram showing nonrecordable scotopic and photopic responses

Figure 6

(a) Humphrey visual fields 30-2 showing visual field constriction (b) Full-field 120 screening three-zone strategy showing visual field constriction

Figure 7

Serial optical coherence tomography showing reduced cystic spaces with thinning of the inner segment-outer segment junction at the macula, sparing the fovea

Figure 8

Humphrey visual fields 30-2 showing improved retinal sensitivity