Table 3

 Summary of ERG monitoring and follow up in the 18 patients; nine of 10 patients showed improvement in full field ERGs following systemic treatment

CaseEDD visitsTreatmentVA before treatmentVA after treatmentPERG P50 changesFull field ERG changesClinical changes
15 (19)Oral prednisolone6/9 6/126/6 6/6See textSee textDeveloped pale discs, vessel sheathing and early lens opacities over 2 years following treatment. VA stable
24 (16)Oral prednisolone6/9 6/96/12 6/5See textSee textMild clinical improvement
311 (43)Oral prednisolone and cyclosporin6/12 6/66/9 6/6See textSee textTreatment improved inflammatory signs but had little impact on VA or CMO. Subjectively and clinically stable
410 (82)Oral prednisolone (1 course)6/9 6/96/6 6/9Initial deterioration then some fluctuationDeteriorationPrednisolone withdrawn due to systemic complications. Gradual mild worsening of VA over 4 years, vascular sheathing
55 (70)DeteriorationDeterioration REMild subjective worsening of vision LE>RE. Pale spots manifest 2.5 years after presentation
63 (15)Oral prednisolone and azathioprine6/9 6/96/9 6/6Marked improvement both eyes followed by deterioration as medication was reducedImproved following treatment but mild deterioration as medication was taperedSubjective improvement in VA. Vasculitis and CMO improved but recurrent inflammation and epiretinal membranes developed as medication was tapered.
73 (16 years)Oral prednisolone 15 years earlierSubnormal 15 years earlier. Undetectable over the past 16 monthsProfound deterioration over 15 years but stable over the past 16 monthsIncreasing annular scotoma and extensive chorioretinal degeneration over 15 years with sparing of central maculae, now stable
84 (67)Orbital floor injections of triamcinolone then oral prednisolone6/60 6/126/24 6/9Subnormal but stable with orbital floor injections. Marked improvement with oral prednisolone, associated with reduced CMOSlight deterioration while being treated with orbital floor injections. Marked improvement with oral prednisoloneOrbital 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.
93 (18)Not treatedImprovedStableMild subjective worsening of vision. Disc oedema and vasculitis improved without treatment. Developed inferior bilateral atrophic scars
104 (33)Oral prednisolone and cyclosporin6/9 6/96/5 6/6Subnormal but stable in the presence of persistent CMOImproved initially, then deterioration as treatment was taperedImprovement 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
111Not treatedMild deterioration in VA over 34 months, Developed photopsias in LE, otherwise stable.
121Topical BetamethasoneWorsening VA over 3 years. Clinically stable over 10 months
133 (38)Oral prednisolone and cyclosporin6/12 6/126/9 6/9Improvement associated with reduced CMOImprovedVA 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
141Oral prednisolone and cyclosporin6/5 6/56/6 6/5Gradual 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
153 (36)Oral prednisolone6/18 6/246/12 6/12UndetectableMild improvement. Deterioration since medication was taperedGradual 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.
161Oral prednisolone (1 course) Depot steroids6/9 6/96/9 6/9Prednisolone 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
173 (71)Systemic treatment refused by patient. Orbital floor steroidsStableSignificant ERG deteriorationFluctuating 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
183 (11)Oral prednisolone6/9 6/126/6 6/9ImprovedMild improvementMild improvement in VA but some fluctuation in inflammatory signs when medication was tapered, stable over past year