The Clinical Characteristics of Unilateral Placoid Pigment Epitheliopathies

Isil Kutluturk, Aniruddha Agarwal, Shiri Shulman, Albert Vitale, Maurizio B. Parodi, Christoph D. Conrady, Katarina Hurtikova, Eduardo Cunha Souza, Ever Caso Rodriguez, Jay Chhablani, Christoph Tappeiner, Heinrich Gerding, Martin S. Zinkernagel, William Tucker, Marion R. Munk*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Purpose: Placoid-pigment-epitheliopathies like Acute-Posterior-Multifocal-Placoid-Pigment Epitheliopathy (APMPPE) and Relentless-Placoid-Choroidopathy (RPC) usually show bilateral involvement. This retrospective case series describes the clinical features with unilateral disease onset. Methods: 21 patients from 9 tertiary referring institutions were collected and evaluated. Results: Seventeen patients were included: 11 (65%) had unilateral placoid-pigment-epitheliopathy consistent with APMPPE, 6 (35%) with RPC (mean age: 28.82 ± 9.1 years). Mean follow-up time was 45 ± 48 months. Forty-one percent fellow-eyes had initial inflammatory signs including vitreous-cells, vascular-sheathing, and ONH swelling. Thirty-five percent developed chorioretinal lesions during follow-up after 19.5 ± 16.9 months. Initial inflammation in fellow-eye was not associated with fellow-eye involvement (p =.5). However, subfoveal choroidal-thickness (SCT) at baseline was significantly thicker in fellow-eyes who developed chorioretinal lesion compared to fellow-eyes who did not (400 ± 70 µm versus 283 ± 127 µm,p =.047). Conclusion: Unaffected fellow-eyes may have inflammatory signs at initial disease-onset and may develop lesions over time. They should be carefully monitored, particularly if they present with an increased SCT.

Original languageEnglish
Pages (from-to)1072-1079
Number of pages8
JournalOcular Immunology and Inflammation
Issue number6
StatePublished - 2021
Externally publishedYes


  • Choroiditis
  • choroidopathy
  • inflammation
  • placoid
  • relentless


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