Coussa Razek Georges, Antaki Fares, Lederer David E
Department of Ophthalmology, McGill Academic Eye Centre, 5252 Boulevard de Maisonneuve West, 4th Floor, Montréal, Québec H4A 3S5, Canada.
Am J Ophthalmol Case Rep. 2018 Jan 28;10:18-24. doi: 10.1016/j.ajoc.2018.01.024. eCollection 2018 Jun.
To report the longest ophthalmic follow-up and the associated ocular complications of HLA-A29 negative Birdshot-like chorioretinopathy (BLCR) associated with common variable immunodeficiency (CVID).
A 22-year-old man known for CVID presented with a 3-month history of decreased visual acuity OS. Funduscopy revealed significant cystoid macular edema OS, as well as optic disk edema and chorioretinal infiltrates without signs of vitritis OU. No infectious, inflammatory or neoplastic etiologies were identified. He subsequently received one dose of intravitreal triamcinolone OS which completely resolved the macular edema. The optic nerve edema persisted despite the addition of intravenous immunoglobulin. His visual acuity was 20/20 OU at the 24th follow-up month.
and importance: To our knowledge, this is the third case of HLA-A29 negative BLCR associated with CVID. It is the first case with long-term follow-up providing, in consequence, the best understanding of the natural history and possible complications of this rare disease. Aggressive systemic treatment, in collaboration with an immunologist, is generally needed to control the ophthalmic complications.
报告与常见可变免疫缺陷（CVID）相关的HLA - A29阴性鸟枪样脉络膜视网膜病变（BLCR）的最长眼科随访情况及相关眼部并发症。
一名已知患有CVID的22岁男性，左眼视力下降3个月。眼底检查发现左眼黄斑囊样水肿明显，以及视盘水肿和脉络膜视网膜浸润，双眼均无葡萄膜炎体征。未发现感染、炎症或肿瘤病因。随后他接受了一次左眼玻璃体内注射曲安奈德，黄斑水肿完全消退。尽管加用了静脉注射免疫球蛋白，视神经水肿仍持续存在。在第24个月随访时，他的双眼视力均为20/20。
据我们所知，这是第三例与CVID相关的HLA - A29阴性BLCR病例。这是首例进行长期随访的病例，因此对这种罕见疾病的自然病程和可能的并发症有了最好的了解。通常需要与免疫学家合作进行积极的全身治疗，以控制眼部并发症。
