Why birdshot retinochoroiditis should rather be called ‘HLA-A29 uveitis’?

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. 2 Ryan and Maumenee first described the bilateral retinochoroidal inflammatory disease in early 1980, reporting on 13 patients. 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. In 1981, Gass described 11 similar cases and called the disease vitiliginous chorioretinitis. 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. 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 below). Retinal involvement was accounted for by calling the disease a retinochoroiditis or a chorioretinitis. Often the disease was also called a retinochoroidopathy or a chorioretinopathy, which was inadequate because it is clearly inflammatory, so that the suffix ‘-itis’ should have been used.