Progressive Expansion of Chorioretinal Atrophy in Placoid-Spectrum Disease

PURPOSE: Chorioretinal atrophy is a prominent feature of placoid-spectrum disorders including acute posterior multifocal placoid pigment epitheliopathy (APMPPE), relentless placoid (ampiginous) chorioretinitis (RPC), and serpiginous choroiditis (SC). The rates of atrophy across different placoid disorders and the evolution of atrophy, including with disease quiescence, remain poorly understood. METHODS: A retrospective chart review of patients with placoid-spectrum disease was performed, with analysis of multimodal imaging for the presence of chorioretinal atrophy (RPE/outer retinal atrophy) at each visit. If the patient achieved clinical disease quiescence, qualitative and quantitative analysis was performed to assess whether the atrophy expanded with time in the absence of ongoing disease activity. RESULTS: A total of 17 patients (5 APMPPE, 12 RPC/SC) were included in the study. Mean age was 26.4 years for APMPPE and 33.8 years for RPC/SC, with average follow up of 13 months and 65 months, respectively. Chorioretinal atrophy was found in 10 (83.3%) patients with RPC/SC and 1 (20%) patient with APMPPE. Chorioretinal atrophy expanded with time despite disease quiescence, with a mean expansion of 0.09 mm/year. CONCLUSION: Chorioretinal atrophy is more often seen in RPC/SC disease than with APMPPE. Atrophic chorioretinal lesions may expand, even when the primary disease is quiescent on immunomodulatory therapy.