Structural and functional changes in non-paraneoplastic autoimmune retinopathy

Abstract

Purpose : Nonparaneoplastic autoimmune retinopathy (npAIR) is an insidious disease that is difficult to monitor due to often-unremarkable findings in clinical exam; therefore, it might be beneficial to use ancillary imaging/tests to evaluate the disease state. We conducted the index case-series to describe ocular changes in patients with npAIR and to assess the utility of different imaging modalities/tests in monitoring the disease course.

Methods : Eight patients (16 eyes) from a tertiary eye care center, with a verified diagnosis of npAIR using the criteria proposed by Sen (Sen et al., 2014), were recruited. Multimodal testing using functional [Goldmann perimetry (GVF), microperimetry (MP), and electrophysiologic testing (ff-ERG, mf-ERG, VEP)] and structural [wide angle fundus photography (WAFP), wide angle fundus autofluorescence (WAFAF), wide angle fluorescein angiography (WAFA), spectral domain optical coherence tomography (SD-OCT), and adaptive optics scanning laser ophthalmoscopy (AOSLO)] tests were performed at different time points and results were analyzed.

Results : The median age of patients was 46.0 [15.5-66.7] years and 50% were female. Initial results showed frank abnormalities in both functional (14 of 16 eyes on GVF, 15 of 16 eyes on MP, 11 of 16 eyes on ff-ERG, 12 of 14 eyes on mf-ERG, 14 of 14 eyes on VEP) tests and structural (14 of 16 eyes on WAFP, 14 of 16 eyes on WAFAF, 13 of 16 eyes on SD-OCT, 5 of 7 eyes on AOSLO) imaging. WAFA appeared normal except in cases of transient macular edema (3 of 16). Subjects were followed up for the median duration of 11.5 [3.0-18.7] months. Follow up imaging of GVF (8 of 10 eyes), mfERG (4 of 8 eyes), and MP (7 of 16 eyes) demonstrated changes which were not always consistent with each other. Ff ERG (9 of 10 eyes) and VEP (7 of 8 eyes) remained mostly unchanged. No significant changes were noted on WAFP and WAFAF. Measurable changes were noted in 4 of 12 eyes on SD-OCT and 6 of 6 eyes on AOSLO. Decrease in retinal sensitivity on MP often preceded subjective symptoms.

Conclusions : Multimodal testing in npAIR patients is crucial to identify and follow changes over time. Deterioration, especially in severe cases, is often subtle, but ongoing as shown by changes in multiple modalities. Most structural modalities might not be able to measure changes once npAIR have become severe. Functional modalities might be able to detect early changes that precede subjective worsening.

This is a 2021 ARVO Annual Meeting abstract.