Purpose

To assess the efficacy of treatment on the short-term outcomes of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and the related entity, relentless placoid chorioretinopathy (RPC).

Methods

We conducted a retrospective case series at three tertiary uveitis centres between December 2016 to November 2020. Identified cases of APMPPE and RPC meeting diagnostic criteria were included in the study. Cases were compared based on treatment received, visual acuity outcomes, lesion incidence during a 6-month study period and optical coherence tomography (OCT) lesion development.

Results

12 patients (9 with bilateral disease) met the diagnostic criteria. 10 patients (17 eyes) had APMPPE. 2 patients (4 eyes) had a clinical course consistent with RPC. 6/12 patients were female. Median age at presentation was 26.5 years (range, 20-57 years). 4 cases (6 eyes) were observed. 10 cases (17 eyes) received corticosteroids +/- second line immunosuppression. Visual gains occurred in both observed and treated cases. Observed bacillary layer detachment (BALAD) and non-BALAD APMPPE lesions achieved favourable anatomical outcomes than comparable treated lesions. Variability was observed in the extent of retinal lesions at presentation and new lesions post presentation. No additional lesions occurred in 6 of 7 observed eyes. Progressive disease was observed in 7 of 10 treated cases. Cases treated with three daily 1g IVMP demonstrated the greatest occurrence of new lesions post-presentation. A delayed, rebound lesion occurrence was observed post-initiation of high dose corticosteroid bilaterally in 3 cases.

Conclusions

Although limited by case numbers, we find no evidence to support the use of corticosteroid treatment in APMPPE or RPC based on this case series.