Table 1.
Clinical characteristics of npAIR patients
| Case#/Sex/Age (y) | Symptoms onset before confirmed dx (m) | Other autoimmune disorder(s) | Total Follow-up duration (y,m) | WB bands at presentation (kDa) | npAIR/RP | BCVA OS/OD at presentation | Immunosuppressors before rituximab/Response before failure |
|---|---|---|---|---|---|---|---|
| 1/F/61 | 24 | Psoriasis, HypoTSH | 6y9m | 33, 45, 55, 64, 72, 90 | Yes/No | 20/150 20/150 |
MMF, I/Improved and stabilized × 60 months on R |
| 2/M/65 | 28 | Cutaneous LE | 4y3m | 40, 46 (enolase), 68 | Yes/Yes | 20/40 20/40 |
MMF/No response after 7-month trial |
| 3/F/16 | 36 | None | 2y10m | 23 (HSP27), 28, 34, 36, 39, 46 (enolase), 62 | Yes/Yes | 20/40 20/40 |
None |
| 4/F/10 | 12 | Crohn Disease | 2y2m | 28, 92 | Yes/No | 20/150 20/125 |
C, P, MMF, IVIG/Improved × 5 months |
| 5/F/70 | 6 | Hashimoto Thyroiditis, Sjogren syndrome | 4y6m | 42 | Yes/No | 20/25 20/30 |
MMF, P/Initial improvement, than compliance variable with stepwise decrease in visual function |
Y years, m months, kDa kilodalton, WB western blot, BCVA best corrected visual acuity, HypoTSH hypothyroidism, Cutaneous LE Cutaneous Lupus Erythematosus, MMF mycophenolate mofetil, I infliximab, R rituximab, C cyclosporine, P prednisone, IVIG intravenous immunoglobulins