Table 1.
Baseline characteristics of the suspected AIR patients.
| Variable | Frequency (percentage) | |
|---|---|---|
| Age of onset (years) | < 20 | 1/44 (2%) |
| 20–40 | 11/44 (25%) | |
| 41–60 | 22/44 (50%) | |
| > 60 | 10/44 (23%) | |
| Gender | Female | 33/44 (75%) |
| Male | 11/44 (25%) | |
| Color vision | Normal | 26/44 (59%) |
| Total color blindness | 6/44 (14%) | |
| Partial color blindness | 1/44 (2%) | |
| Cannot be evaluated due to low vision | 9/44 (21%) | |
| N/A | 2/44 (5%) | |
| ERG | Reduced rod and cone responses | 9/44 (21%) |
| Reduced cone response, normal rod response | 1/44 (2%) | |
| Reduced rod response, normal cone response | 1/44 (2%) | |
| Absence of rod response, reduced cone response | 1/44 (2%) | |
| Asymmetrical | 1/44 (2%) | |
| Nonrecordable | 29/44 (66%) | |
| N/A | 2/44 (5%) | |
| Clinical symptoms | Blurred vision | 23/44 (52%) |
| Nyctalopia | 29/44 (66%) | |
| Reduction in peripheral visual field | 19/44 (43%) | |
| Othersa | 5/44 (11%) | |
| No symptoms | 2/44 (5%) | |
| Relevant underlying condition | Autoimmune diseaseb | 12/44 (27%) |
| Malignancy | 2/44 (5%) | |
| Family history of autoimmune disease | 2/44 (5%) | |
| None | 29/44 (66%) | |
| BCVA (logMAR, eyes) | Good (< 0.54) | 56/87 (64%) c |
| Moderate (0.54–1.00) | 12/87 (14%) c | |
| Legally blind (≥ 1.00) | 19/87 (22%) c | |
| Visual field (eyes)d | Reduced < 50% from normal visual field | 13/79 (17%) |
| Reduced > 50% from normal visual field | 66/79 (84%) | |
| Central scotoma | 3/79 (4%) | |
| Paracentral scotoma | 22/79 (28%) | |
| Cannot be evaluated (eyes) | 8/87 (9%) c | |
| Intraocular lens (eyes) | 28/87 (32%) c | |
| Previous medications | Chloroquine or hydroxychloroquine | 5/44 (11%) |
BCVA best corrected visual acuity, N/A not applicable.
aPhotoaversion, photosensitivity, photopsia, hemeralopia, eye pain, dry eye.
bSystemic lupus erythematosus (SLE), rheumatoid arthritis (RA), pemphigus vulgaris, minimal change disease (MCD), undifferentiated connective tissue disease (UCTD).
cOne patient had one prosthetic eye.
dThere were two patients without VF data and five patients with VF data only in one eye.