Cancer-associated retinopathy (CAR) is a potentially blinding disease triggered by autoimmunity to cancer antigens at distant sites. It may masquerade as immune-related adverse events from the use of immune checkpoint inhibitors (ICIs). We present a patient with an underlying tubby-related protein 1 (TULP1) cancer-associated retinopathy who lost vision following initiation of atezolizumab for small-cell lung cancer. This 75-year-old man presented with no light perception, paramacular and peripheral retinal pigmentary changes, attenuated outer retina, and extinguished rod and cone responses. The visual loss followed the induction of atezolizumab therapy. Possible atezolizumab-associated acute macular neuroretinopathy was considered, and atezolizumab was discontinued. Vision improved on oral corticosteroid and deteriorated when corticosteroid was tapered quickly. Retinal autoantibody serology testing was negative for both anti-recoverin and anti-enolase and was positive for anti-TULP1 autoantibodies. Re-induction of atezolizumab concomitant with high-dose oral and intravitreal corticosteroids resulted in visual recovery at the three-month follow-up. These findings suggest that ICI therapy for cancer can exacerbate the retinal dysfunction in a patient with underlying autoimmunity from cancer. Patients with a high risk of CAR may need to be evaluated for retinal autoantibodies before initiation of ICI.
Keywords: cancer-associated retinopathy; paraneoplastic; programmed death ligand-1; tulp1; vision loss.
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