Menezo Victor, Taylor Simon Rj
Institut Catala de Retina, Barcelona, Spain ; Department of Ophthalmology, Provincial Hospital Consortium Castellon, Castello, Spain.
Faculty of Medicine, Imperial College London, Hammersmith Hospital, London, UK ; Royal Surrey County Hospital NHS Foundation Trust, Guildford, UK.
Clin Ophthalmol. 2014;8:73-81. doi: 10.2147/OPTH.S54832. Epub 2013 Dec 18.
Birdshot chorioretinopathy is a relatively uncommon subtype of idiopathic posterior uveitis with distinct clinical characteristics and a strong genetic association with the Human Leukocyte Antigen (HLA)-A29 allele. The diagnosis remains clinical and is based on the presence of typical clinical features, including multiple, distinctive, hypopigmented choroidal lesions throughout the fundus. The long-term visual prognosis of this disorder, however, remains guarded - central visual acuity can be preserved until late in the disease and it is not uncommon for patients to receive inadequate immunosuppressive treatment, leading to a poor long-term outcome in which peripheral retinal damage eventually leads to visual deterioration. Birdshot chorioretinopathy has proven a particularly attractive area of study within the field of uveitis, as it is a relatively easily defined disease with an associated human leukocyte antigen haplotype. Despite this, however, the immune mechanisms involved in its pathogenesis remain unclear, and some patients continue to lose retinal function despite therapy with corticosteroids and conventional immunosuppressive agents. Laboratory research continues to investigate the underlying mechanisms of disease, and clinical research is now being driven to improve the phenotyping and monitoring of this condition as, in the era of so-called personalized medicine, it is becoming increasingly important to identify patients at risk of visual loss early so that they can be treated more aggressively with targeted therapies such as the newer biological agents. This approach requires the formation of collaborative groups, as the relative rarity of the condition makes it difficult for one center to accumulate enough patients for worthwhile studies. Nevertheless, results obtained with newer therapies, such as biological agents directed against particular cytokines or cell-surface receptors, demonstrate ever improving control of the inflammation in refractory cases, providing hope that the outlook for visual function in this condition can only improve.
鸟枪弹样脉络膜视网膜病变是特发性后葡萄膜炎中一种相对不常见的亚型，具有独特的临床特征，并且与人类白细胞抗原（HLA）-A29等位基因有很强的遗传关联。诊断仍基于临床，依据典型临床特征的存在，包括眼底出现多个独特的色素脱失性脉络膜病变。然而，这种疾病的长期视力预后仍不容乐观——中心视力可在疾病晚期得以保留，患者接受不足的免疫抑制治疗并不罕见，这会导致不良的长期结局，即周边视网膜损伤最终导致视力恶化。鸟枪弹样脉络膜视网膜病变已被证明是葡萄膜炎领域中一个特别有吸引力的研究领域，因为它是一种相对容易定义的疾病，且有相关的人类白细胞抗原单倍型。尽管如此，其发病机制中涉及的免疫机制仍不清楚，一些患者尽管接受了皮质类固醇和传统免疫抑制剂治疗，仍继续丧失视网膜功能。实验室研究继续探究疾病的潜在机制，临床研究目前正朝着改善这种疾病的表型分析和监测发展，因为在所谓的个性化医疗时代，尽早识别有视力丧失风险的患者变得越来越重要，以便他们能够接受更新的生物制剂等靶向治疗更积极的治疗。这种方法需要组建协作组，因为该疾病相对罕见，一个中心难以积累足够数量的患者进行有价值的研究。尽管如此，针对特定细胞因子或细胞表面受体的生物制剂等新疗法所取得的结果表明，难治性病例中的炎症控制不断改善，这为改善这种疾病的视觉功能前景带来了希望。
