title: Birdshot chorioretinopathy: current knowledge and new concepts in pathophysiology, diagnosis, monitoring and treatment.
abstract: Birdshot chorioretinopathy (BCR) is a rare form of chronic, bilateral, posterior uveitis with a distinctive clinical phenotype, and a strong association with HLA-A29. It predominantly affects people in middle age. Given its rarity, patients often encounter delays in diagnosis leading to delays in adequate treatment, and thus risking significant visual loss. Recent advances have helped increase our understanding of the underlying autoimmune mechanisms involved in disease pathogenesis, and new diagnostic approaches such as multimodality imaging have improved our ability to both diagnose and monitor disease activity. Whilst traditional immunosuppressants may be effective in BCR, increased understanding of immune pathways is enabling development of newer treatment modalities, offering the potential for targeted modulation of immune mediators. In this review, we will discuss current understanding of BCR and explore recent developments in diagnosis, monitoring and treatment of this disease. Synonyms for BCR: Birdshot chorioretinopathy, Birdshot retinochoroiditis, Birdshot retino-choroidopathy, Vitiliginous choroiditis. Orphanet number: ORPHA179 OMIM: 605808.
authors.affiliation: Department of Ophthalmology, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
authors.forename: Evangelos
authors.initials: E
authors.lastname: Minos
authors.affiliation: Department of Ophthalmology, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.|Academic Unit of Ophthalmology, Centre for Translational Inflammation Research, University of Birmingham, Birmingham, West Midlands, B15 2WB, UK.|Birmingham & Midland Eye Centre, Sandwell & West Birmingham Hospitals NHS Trust, Birmingham, UK.
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Barry
authors.affiliation: Department of Ophthalmology, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.|Birdshot Uveitis Society, London, UK.
authors.forename: Sue
authors.initials: S
authors.lastname: Southworth
authors.affiliation: Birdshot Uveitis Society, London, UK.
authors.forename: Annie
authors.initials: A
authors.lastname: Folkard
authors.affiliation: Academic Unit of Ophthalmology, Centre for Translational Inflammation Research, University of Birmingham, Birmingham, West Midlands, B15 2WB, UK.|Birmingham & Midland Eye Centre, Sandwell & West Birmingham Hospitals NHS Trust, Birmingham, UK.
authors.forename: Philip I
authors.initials: PI
authors.lastname: Murray
authors.affiliation: New England Eye Center, Tufts Medical Center, Tufts University School of Medicine, Boston, USA.
authors.forename: Jay S
authors.initials: JS
authors.lastname: Duker
authors.affiliation: NIHR Biomedical Research Centre for Ophthalmology, Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology, London, United Kingdom.
authors.forename: Pearse A
authors.initials: PA
authors.lastname: Keane
authors.affiliation: Department of Ophthalmology, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK. a.denniston@bham.ac.uk.|Academic Unit of Ophthalmology, Centre for Translational Inflammation Research, University of Birmingham, Birmingham, West Midlands, B15 2WB, UK. a.denniston@bham.ac.uk.|Birmingham & Midland Eye Centre, Sandwell & West Birmingham Hospitals NHS Trust, Birmingham, UK. a.denniston@bham.ac.uk.
authors.forename: Alastair K
authors.initials: AK
authors.lastname: Denniston
mesh_terms: D000080365:Birdshot Chorioretinopathy; D002825:Chorioretinitis / Q000175:diagnosis* / Q000378:metabolism / Q000503:physiopathology* / Q000628:therapy; D015234:HLA-A Antigens / Q000378:metabolism; D006801:Humans; D056747:Immunomodulation; D058504:Th17 Cells / Q000276:immunology
chemical_list: D015234:HLA-A Antigens; C035524:HLA-A29 antigen
keywords: Birdshot chorioretinopathy; HLA-A29; Immunomodulatory therapy; Multimodality diagnostic imaging; T-helper 17