Birdshot Chorioretinopathy: A Review.

Area of Science:

• Ophthalmology
• Immunology
• Genetics

Background:

• Birdshot chorioretinopathy (BSCR) is a chronic, bilateral inflammatory eye disease.
• It predominantly affects middle-aged individuals of European descent and is strongly associated with the human leucocyte antigen (HLA)-A29 allele.
• The precise immune mechanisms underlying BSCR are still being elucidated, with recent focus on Endoplasmic Reticulum Aminopeptidase 2 (ERAP2).

Purpose of the Study:

• To provide an updated review of birdshot chorioretinopathy.
• To discuss current understanding of its pathophysiology, diagnostic imaging modalities, and therapeutic strategies.

Main Methods:

• Review of existing literature on birdshot chorioretinopathy.
• Analysis of diagnostic utility of multimodal imaging techniques.
• Evaluation of current treatment approaches, including corticosteroids and immunosuppressive/biological therapies.

Main Results:

• The human leucocyte antigen (HLA)-A29 allele is a key genetic factor in BSCR.
• Endoplasmic Reticulum Aminopeptidase 2 (ERAP2) is implicated in the disease's pathogenesis.
• Multimodal imaging (fluorescein angiography, indocyanine angiography, fundus autofluorescence, optical coherence tomography) and visual field testing are crucial for diagnosis and monitoring.
• Optimal treatment remains debated, with a need for long-term immunosuppressive or biological therapies.

Conclusions:

• Birdshot chorioretinopathy pathogenesis involves complex immune mechanisms, including ERAP2.
• Accurate diagnosis and disease monitoring rely on advanced multimodal imaging and visual field testing.
• Long-term management necessitates immunosuppressive or biological agents due to the chronic nature of the disease.