Spectral domain optical coherence tomography as an effective screening test for hydroxychloroquine retinopathy (the “flying saucer” sign)

Figures & data

Table 1 Patient demographics and results

Patient	Age	Sex	Dx	HCQ (mg/d)	HCQ (mg/kg/d)	Rx (years)	Total dose (g)	Symptoms	VA	HVF 10-2	Fundus	SD OCT changes
1	45	M	LPP	400	4.5	<1	NA	None	20/20, 20/20	Normal	Normal	No
2	57	F	SCL	400	7.2	15	2190	None	20/25, 20/20	Scattered defects	Normal	No
3	64	F	DM	400	5.4	4	584	None	20/20, 20/30	Normal	Normal	No
4	47	F	RA	400	8.4	2	292	Dec VA	20/30, 20/30	Paracentral scotoma OU	Early bull’s eye	Yes
5	38	F	SLE	400	NA	<1	NA	None	20/20, 20/25	Normal	RPE changes	No
6	52	F	SLE	400	7.0	5	730	None	20/20, 20/20	Few nonspecific changes	Normal	No
7	28	F	SLE	400	5.4	3	438	None	20/20, 20/25	Normal	Normal	No
8	66	F	SLE	400	7.2	9	1314	None	20/20, 20/20	Normal	Mild RPE changes	No
9	67	F	SLE	400	5.8	5	730	None	20/25, 20/25	Paracentral scotoma OD > OS	RPE changes	Yes
10	50	F	SS	400	8.0	8	1168	None	20/20, 20/20	Paracentral scotoma OU	Normal	Yes
11	59	F	SLE, RA	400	4.8	18	2628	Scotoma OS	20/20, 20/20	Central OS > OD	Normal	No
12	45	F	RA	400	4.6	3	438	None	20/20, 20/20	Normal	Mild RPE changes	No
13	40	F	SLE	400	5.5	<1	NA	None	20/20, 20/20	Normal	Normal	No
14	78	F	RA	200	4.2	4	292	None	20/50, 20/30	Mild paracentral changes OU	RPE changes	No
15	50	F	SLE	400	3.7	<1	NA	Dec VA	20/25, 20/30	Paracentral OS > OD	Mild MP OS	No
16	64	F	ANA +	200	3.0	10	730	None	20/20, 20/20	Normal	Mild RPE changes	No
17	60	F	LPP	400	6.1	1	146	Dec VA	20/30, 20/30	Nonspecific changes, OS	Few drusen	No
18	52	F	SLE	400	5.5	20	2920	Dec VA	HM, CF	Dense scotoma	Bull’s eye mac	Yes
19	61	F	SLE	400	4.6	3	438	None	20/20, 20/20	Early scotoma OD	Normal	No
20	63	F	RA	400	6.8	17	2482	None	20/20, 20/25	Few nonspecific changes	Normal	No
21	38	F	SS	400	7.1	1	146	None	20/25, 20/20	Normal	Normal	No
22	40	F	SLE	200	3.9	22	1606	None	20/20, 20/20	Normal	Normal	No
23	68	F	LPP	400	5.7	<1	NA	None	20/25, 20/30	Normal	Normal	No
24	78	F	SLE	200	2.9	9	657	Dec VA	20/40, 20/30	Paracentral scotoma OU	Bull’s eye mac	Yes
25	60	F	RA	400	5.7	10	1460	None	20/20, 20/20	Normal	Mild MP OS	No

Abbreviations: CF, count fingers; DM, dermatomyositis; F, female; HCQ, hydroxychloroquine; HM, hand motion; HVF, Humphrey visual field; LLP, lichen planopilaris; M, male; MP, macular pucker; NA, not applicable; RA, rheumatoid arthritis; SCL, scleroderma; SD OCT, spectral domain optical coherence tomography; SLE, systemic lupus erythematosus; SS, Sjogren’s syndrome. OS, outer segment; RPE, retinal pigment epithelium; VA, visual acuity.

Figure 1 Top: Normal Spectralis spectral domain optical coherence tomography (SD OCT) image with intact photoreceptor inner segment/outer segment junction (IS/OS). Bottom: Spectralis SD OCT from the left eye of patient 10 showing the “flying saucer” sign of hydroxychloroquine retinopathy, an ovoid appearance of the central fovea created by preservation of central foveal outer retinal structures (seen between the black arrows) surrounded by perifoveal loss of the photoreceptor IS/OS junction, and perifoveal outer retinal thinning.

Abbreviations: ILM, internal limiting membrane; IPL, inner plexiform layer; OPL, outer plexiform layer; ELM, external limiting membrane; RPE, retinal pigment epithelium.

Figure 2 Spectral domain optical coherence tomography (SD OCT) images taken with different commercially available SD OCT machines demonstrate the “flying saucer” sign is consistent in different individuals with hydroxychloroquine retinopathy. A) Heidelberg Spectralis SD OCT in patient 9, OD. B) Zeiss Cirrus SD OCT in patient 9, OD. C) Heidelberg Spectralis SD OCT in patient 4, OS. D) Zeiss Cirrus SD OCT in patient 4, OS.

Figure 3 Spectral domain optical coherence tomography (SD OCT), red-free photo, fundus autofluorescence, and early and late fluorescein angiogram in patient 10, who had no symptoms, normal clinical exam and fundus autofluorescence imaging, Humphrey visual field 10-2 with paracentral scotomas, and “flying saucer” sign seen on SD OCT.

Figure 4 Spectral domain optical coherence tomography (SD OCT) images, red-free photos, and fundus autofluorescence (FAF) demonstrate a spectrum of findings seen with different stages of hydroxychloroquine retinopathy. Note the increased signal of choroidal vessels on SD OCT as the retina becomes increasingly atrophic with progressive toxicity starting from early toxicity in patient 10 seen in Figure 3. A) Moderate toxicity. Patient 24: vision loss, clinical bull’s eye maculopathy even more apparent on FAF, Humphrey visual field (HVF) 10-2 paracentral scotomas, and significant perifoveal outer retinal dropout but preservation of central photoreceptor inner segment/outer segment junction (between the arrows) seen on SD OCT allowing 20/40 vision. B) Late toxicity. Patient 18: severe vision loss to hand motion, pronounced clinical and FAF bull’s eye maculopathy, dense HVF 10-2 paracentral scotomas, and complete disruption of outer retinal structures on SD OCT.