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Why birdshot retinochoroiditis should rather be called ‘HLA-A29 uveitis’?
  1. Carl P Herbort Jr1,2,
  2. Carlos Pavésio3,
  3. Phuc LeHoang4,
  4. Bahram Bodaghi4,
  5. Christine Fardeau4,
  6. Philippe Kestelyn5,
  7. Piergiorgio Neri6,
  8. Marina Papadia1,7
  1. 1Retinal and Inflammatory Eye Disease, Centre for Ophthalmic Specialized Care (COS), Clinic Montchoisi Teaching Centre, Lausanne, Switzerland
  2. 2University of Lausanne, Lausanne, Switzerland
  3. 3National Institute for Health Research Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust and UCL Institute of Ophthalmology, London, UK
  4. 4Department of Ophthalmology, DHU View Maintain, La Pitié Salpêtrière University Hospital, Université Pierre et Marie Curie, Paris, France
  5. 5Department of Ophthalmology, University of Ghent, Ghent, Belgium
  6. 6Department of Clinical and Experimental Medicine, Ocular Immunology Unit, The Eye Clinic, Ospedali Riuniti di Ancona, Polytechnic University of Marche-Ancona, Ancona, Italy
  7. 7Department of Ophthalmology, Ospedale Padre Antero Micone, Genova, Genova, Italy
  1. Correspondence to Dr Carl P Herbort Jr, Retinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), Rue Charles-Monnard 6, Lausanne CH-1003, Switzerland; cph{at}herbortuveitis.ch

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Birdshot retinochoroiditis: the origins of the disease name

Birdshot retinochoroiditis (BRC) is a uveitis predominantly affecting the posterior segment of the eye with dual, independent retinal and choroidal inflammation and almost only seen in Caucasians.1 ,2 Ryan and Maumenee first described the bilateral retinochoroidal inflammatory disease in early 1980, reporting on 13 patients.2 These authors called it ‘birdshot retinochoroidopathy’ because they found that lesions had a pattern comparable to that seen in wounds produced by birdshot shotguns. Later in 1980, Kaplan and Aaberg published a series of four similar cases ‘involving a choroidal and retinal pigment epithelium disease not previously described’, which they also called birdshot retinochoroidopathy.3 In 1981, Gass described 11 similar cases and called the disease vitiliginous chorioretinitis.4

In these first articles on the disease, clinicians had no choice but to rely on fundus examination and fluorescein angiography (FA). Therefore, the striking choroidal involvement seen on fundus examination was at the origin of the eponym the authors chose to give to the disease, because both ‘birdshot’ and ‘vitiliginous’ refer to the typical rice-shaped, depigmented choroidal birdshot fundus lesions (BFLs) that strongly and specifically characterised the condition. Of the two designations, ‘birdshot’ became the universally used and accepted term. It is understandable that such remarkable choroidal signs determined the naming of the disease because the retinal and FA signs were so much less specific and pathognomonic. Nevertheless, an important FA sign was noted by Gass, who observed retinal vasculitis of small and large vessels and the profuse leakage of fluorescein into the retina in the early disease, as well as a reduced presence of fluorescein in the large veins.4 He interpreted this feature as a substantial delay in retinal arteriovenous circulation, an explanation that was later shown to be inexact, thanks to the use of dual FA/indocyanine green angiography (ICGA) (see …

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