TABLE.

Characteristics of Patients With Autoimmune Retinopathy

Patient # (Age, Sex)	Presenting Symptoms	BCVA at Presentation	Fundus Findings	Serum Antiretinal Antibodies	ERG	Macular OCT Findings/Central Macular Thickness	Nerve Fiber Layer Thickness	Associated Autoimmune Disease/Malignancy
1 (85, M)	Progressive loss of vision from 20/60 and 20/25 over 1 year, poor peripheral vision, photopsia	HM OD 20/70 OS	Mild optic nerve head pallor, attenuated vessels, pigment clumping in macula	30 kDa (carbonic anhydrase), 46 kDa (α-enolase) 52 and 67 kDa	Extinguished rod and cone responses	Loss of photoreceptor layer; bilateral epiretinal membrane; 273 μm OD 220 μm OS	Unremarkable	RA
2 (51, F)	Progressive loss of vision over 2 years, photopsia	20/200 OU	Old PRP & focal laser, regressed PDR, attenuated vessels	46 kDa (α-enolase)	Extinguished rod and cone responses	Loss of photoreceptor layer; 92 μm OD 101 μm OS	Moderate thinning inferiorly OD	None
3 (54, F)	Progressive loss of vision over 1 year	20/20-OU	Unremarkable	30 kDa (carbonic anhydrase), 33 kDa	Normal rod, increased implicit time on cone responses	Central macular thinning with no apparent Photoreceptor loss; 176 μm OD 167 μm OS	Mild thinning temporally OU	Lymphoma, breast cancer, cryoglobulinemia, hepatitis C
4 (70, F)	Rapid decline in acuity from 20/30 over 4 months	20/200 OU	Focal RPE atrophy OD, normal OS Central window defects on FA OU	145 kDa (IRBP)	Not available	Loss of photoreceptor layer; 128 μm OD 129 μm OS	Unremarkable	SLE, antiphospholipid antibody syndrome
5 (69, F)	Rapid decline in acuity from 20/40 over 3 months	20/200 OD 20/70 OS	Unremarkable	46 kDa (α-enolase)	Normal rod, significantly reduced cone responses	Loss of photoreceptor layer; 173 μm OD 168 μm OS	Unremarkable	None
6 (49, F)	Central visual loss over 6 months	6/200 E OU	Unremarkable	35–36 kDa and 44 kDa	Delayed and significantly reduced cone and rod responses	Central macular thinning with abnormal foveal depression; mild disruption of photoreceptor OS/IS junction; 151 μm OD 146 μm OS	Unremarkable	None
7 (78, F)	Decreased vision over 6–8 months, photopsia	20/70 OU	Attenuated vessel, scattered RPE changes OU	50 kDa, 62 kDa, and 67 kDa	Extinguished rod and cone responses	Loss of photoreceptor layer; 149 μm OD 147 μm OS	Moderate thinning inferiorly OD; moderate thinning superiorly & inferiorly OS	Ovarian cancer
8 (40, F)	Decreased acuity over 2 months, severely affected visual field on Humphrey 24-2 and 10-2 testing, photopsia OD only	20/70 declined to 1/200 E OD 20/20 OS	Bilateral hypopigmentation of inferior retina	40 kDa	Normal rod and cone responses on full-field ERG; reduced macular response OD on multifocal ERG	Disruption of photoreceptor OS/IS junction; 277 μm OD 206 μm OS	Unremarkable	Graves disease Positive ANA

ANA = antinuclear antibody; BCVA = best-corrected visual acuity; E = 20/200 “E” Snellen optotype; ERG = electroretinogram; F = female; FA = fluorescein angiogram; HM = hand motion; IRBP = interphotoreceptor retinoid-binding protein; M = male; OCT = optical coherence tomography; OS/IS = outer segment/inner segment; PDR = proliferative diabetic retinopathy; PRP = panretinal photocoagulation; RA = rheumatoid arthritis; RPE = retinal pigment epithelium; SLE = systemic lupus erythematosus.