E.NEO1, M.TIEN1, K.CHIA1, G.E.HOLDER1, S.L.HO1, Z.X.THNG1, R.AGRAWAL1, N.GAN1
Tan Tock Seng Hospital1
Autoimmune retinopathy (AIR) is a difficult condition to diagnose that often confounds ophthalmologists. A delay in diagnosis often results in significant morbidity. This case series presents a spectrum of patients with non-paraneoplastic autoimmune retinopathy (npAIR).
Retrospective chart review for patients with posterior uveitis with possible diagnosis of npAIR at a tertiary referral eye care centre in Singapore from 2019 and 2021. Clinical presentation, multimodal imaging and electroretinogram findings were retrieved. Serum anti-retinal antibodies were tested in selected cases. Baseline demographic data, visual acuity, clinical and imaging features, treatment and disease progression were analyzed.
4 patients (80%) were female with a mean age of symptom onset at 39.8 +/- 12.4 years and mean visual acuity of LogMAR 0.260 (Snellen 6/13.2). The most common presenting symptoms were photopsia or visual field loss. Formal visual field testing revealed corresponding visual field defects or enlarged blind spot. Electroretinogram showed generalized photoreceptor dysfunction but with variable severity between the cases. Optical coherence tomography and fundus autofluorescence most commonly showed ellipsoid zone disruption and patchy hyper-autofluorescence respectively. Most patients were treated with a combination of high dose corticosteroids and longer-term steroid-sparing immunosuppression, with most showing stabilization of disease but no structural improvement.
npAIR still remains a challenging disease to diagnose and treat. Without a high index of suspicion, it can be easily misdiagnosed for less sinister conditions. Immunosuppressive therapy can be used to prevent disease progression and contralateral eye involvement. However, further research is needed to determine the best diagnostic and management modalities to offer patients.