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C.P. Herbort, K. Probst, L. Cimino; Differential Involvement and Response to Therapy of Retinal and Choroidal Disease in Birdshot Retinochoroidopathy, a Longitudinal Study . Invest. Ophthalmol. Vis. Sci. 2003;44(13):2387.
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Background. Birdshot retinochoroidopathy is characterised by dual unrelated inflammatory involvement of retina and choroid. Indocyanine green angiography made it possible to assess and follow choroidal disease with the same precision as retinal disease. The aim of this study was to analyse the severity, progression and response to therapy of both retinal involvement using fluorescein angiography (FA) and choroidal involvement using indocyanine green angiography (ICGA). Methods & Patients. Patients with birdshot retinochoroidopathy followed at La Source Eye Centre in Lausanne, Switzerland from January 1995 to November 2002 were subdivided into three subgroups according to the duration of the disease: untreated patients with less than one year disease duration (n=6) ; treated patients from 1-7 years' duration (n=5) and patients with more than 7 years' duration (n=4). Fluorescein and ICGA signs (angiographic scores 0-4 given by a masked observer) were analysed in the 3 groups and compared to morphological parameters (depigmented fundus areas) and to functional parameters (visual field testing). Results Fifteen out of the 742 patients (2.0%) seen at La Source Eye Centre during the time period considered presented BC and were included in the study. In the "early disease group" FA and ICGA showed more severe choroidal than retinal involvement with respective scores of 2 ± 1.17 and 3 ± 0.79 while there were few depigmented fundus areas (score 1 ± 0.27). The choroidal involvement responded well to systemic steroids ± immunosuppressive therapy (scores in groups 2 & 3 = 1.2 and 0.75), while retinal disease was stabilised at best (scores in groups 2 & 3 = 2.2. & 2.4) and depigmented fundus lesions increased (scores in groups 2 & 3 = 2.8 & 3). Visual field parameters improved at the initiation of therapy but then slowly deteriorated again. Conclusion. The evolution and response to therapy of retinal and choroidal disease in birdshot have a different course with choroidal disease responding well to therapy while retinal disease is more resistant, possibly explaining the slow deterioration of functional parameters despite therapy. The increase of depigmented fundus areas despite good choroidal response to therapy could be explained by depigmentation left behind after resolution of choroidal stromal granulomata, a hypothesis recently confirmed by an autopsy case of birdshot retinochoroidopathy.
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