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SPLIT OUTER PLEXIFORM LAYER APPEARANCE REPRESENTS AN ACQUIRED CONE DYSFUNCTION PHENOTYPE OF AUTOIMMUNE RETINOPATHY.

Timothy M Boyce1,2, Christopher Fortenbach1,2, Matthew Thurtell2,3

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Autoimmune retinopathy (AIR) can cause vision loss with a unique OCT finding. Early recognition of this acquired cone dysfunction is key, as treatment may improve or stabilize vision.

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Area of Science:

  • Ophthalmology
  • Immunology
  • Retinal Imaging

Background:

  • Autoimmune retinopathy (AIR) presents a diagnostic challenge due to its varied clinical features.
  • A specific subtype of AIR with a distinct split outer plexiform layer on OCT has been recently identified.

Purpose of the Study:

  • To characterize the clinical presentation of a specific subtype of autoimmune retinopathy.
  • To investigate the association between split outer plexiform layer on OCT and acquired cone dysfunction.

Main Methods:

  • Retrospective observational study of 10 patients diagnosed with AIR.
  • Analysis of clinical data, multimodal imaging (OCT), and electrophysiologic testing.
  • Review of patient outcomes following treatment.

Main Results:

  • All patients experienced bilateral, asymmetric, progressive central vision loss.
  • Fifty percent of patients had a concurrent or subsequent cancer diagnosis.
  • Treatment with immunosuppressives or tumor resection led to partial visual recovery in some patients.

Conclusions:

  • The split outer plexiform layer on OCT is indicative of acquired cone dysfunction in AIR.
  • Prompt diagnosis and treatment of this AIR subtype can potentially improve or stabilize visual function.