Summary of ERG monitoring and follow up in the 18 patients; nine of 10 patients showed improvement in full field ERGs following systemic treatment
| Case | EDD visits | Treatment | VA before treatment | VA after treatment | PERG P50 changes | Full field ERG changes | Clinical changes |
|---|---|---|---|---|---|---|---|
| 1 | 5 (19) | Oral prednisolone | 6/9 6/12 | 6/6 6/6 | See text | See text | Developed pale discs, vessel sheathing and early lens opacities over 2 years following treatment. VA stable |
| 2 | 4 (16) | Oral prednisolone | 6/9 6/9 | 6/12 6/5 | See text | See text | Mild clinical improvement |
| 3 | 11 (43) | Oral prednisolone and cyclosporin | 6/12 6/6 | 6/9 6/6 | See text | See text | Treatment improved inflammatory signs but had little impact on VA or CMO. Subjectively and clinically stable |
| 4 | 10 (82) | Oral prednisolone (1 course) | 6/9 6/9 | 6/6 6/9 | Initial deterioration then some fluctuation | Deterioration | Prednisolone withdrawn due to systemic complications. Gradual mild worsening of VA over 4 years, vascular sheathing |
| 5 | 5 (70) | — | — | — | Deterioration | Deterioration RE | Mild subjective worsening of vision LE>RE. Pale spots manifest 2.5 years after presentation |
| 6 | 3 (15) | Oral prednisolone and azathioprine | 6/9 6/9 | 6/9 6/6 | Marked improvement both eyes followed by deterioration as medication was reduced | Improved following treatment but mild deterioration as medication was tapered | Subjective improvement in VA. Vasculitis and CMO improved but recurrent inflammation and epiretinal membranes developed as medication was tapered. |
| 7 | 3 (16 years) | Oral prednisolone 15 years earlier | — | — | Subnormal 15 years earlier. Undetectable over the past 16 months | Profound deterioration over 15 years but stable over the past 16 months | Increasing annular scotoma and extensive chorioretinal degeneration over 15 years with sparing of central maculae, now stable |
| 8 | 4 (67) | Orbital floor injections of triamcinolone then oral prednisolone | 6/60 6/12 | 6/24 6/9 | Subnormal but stable with orbital floor injections. Marked improvement with oral prednisolone, associated with reduced CMO | Slight deterioration while being treated with orbital floor injections. Marked improvement with oral prednisolone | Orbital floor injections initially reduced ocular inflammation and periphlebitis but RE VA suddenly fell to 6/60. Marked bilateral improvement in vitritis, CMO, and VA on systemic steroids. |
| 9 | 3 (18) | Not treated | — | — | Improved | Stable | Mild subjective worsening of vision. Disc oedema and vasculitis improved without treatment. Developed inferior bilateral atrophic scars |
| 10 | 4 (33) | Oral prednisolone and cyclosporin | 6/9 6/9 | 6/5 6/6 | Subnormal but stable in the presence of persistent CMO | Improved initially, then deterioration as treatment was tapered | Improvement in VA within 2 weeks but with persistent CMO. Inflammatory signs became quiescent but recurred when medication was tapered. Increasing steroids controlled rebound inflammation but disc pallor slowly worsening |
| 11 | 1 | Not treated | — | — | — | — | Mild deterioration in VA over 34 months, Developed photopsias in LE, otherwise stable. |
| 12 | 1 | Topical Betamethasone | — | — | — | — | Worsening VA over 3 years. Clinically stable over 10 months |
| 13 | 3 (38) | Oral prednisolone and cyclosporin | 6/12 6/12 | 6/9 6/9 | Improvement associated with reduced CMO | Improved | VA stable over 15 months but fluctuating signs including CMO, floaters, and mild perceptual disturbance as cyclosporin was tapered. Essentially stable over following 18 months on low dose cyclosporin |
| 14 | 1 | Oral prednisolone and cyclosporin | 6/5 6/5 | 6/6 6/5 | — | — | Gradual deterioration in VA over 3 years. Clearer vitreous following high dose therapy but fresh BCR lesions on right and worsening of vitritis as medication was tapered. Right macula developed RPE changes, posterior vitreous detachment LE |
| 15 | 3 (36) | Oral prednisolone | 6/18 6/24 | 6/12 6/12 | Undetectable | Mild improvement. Deterioration since medication was tapered | Gradual improvement in VA and colour vision and slight reduction in vitritis over 2 years. Intraretinal bleeding associated with high BP. Slight reduction in VA but clinically stable for 2 years since therapy ceased. |
| 16 | 1 | Oral prednisolone (1 course) Depot steroids | 6/9 6/9 | 6/9 6/9 | — | — | Prednisolone withdrawn due to raised BP (no subjective improvement). Periocular depot steroids initially resulted in reduced floaters and vitritis but recurrence of vitritis 2 years later |
| 17 | 3 (71) | Systemic treatment refused by patient. Orbital floor steroids | — | — | Stable | Significant ERG deterioration | Fluctuating VA over 8 years and increased photopsiae. Reported visual disturbances on eye closure. Developed multiple atrophic RPE lesions, macular and peripheral retinal thickening, vaculitis and vascular attenuation |
| 18 | 3 (11) | Oral prednisolone | 6/9 6/12 | 6/6 6/9 | Improved | Mild improvement | Mild improvement in VA but some fluctuation in inflammatory signs when medication was tapered, stable over past year |