PDF(Pubmed)
Abstract：
BACKGROUND: Frosted branch angiitis is a retinal vascular condition that is associated with a viral infection or autoimmune disorders like Crohn\'s disease, systemic lupus erythematosus, and Behcet\'s disease. Frosted branch angiitis presents with vascular inflammation, retinal edema, and severe retinal vascular sheathing. We present a case of systemic juvenile idiopathic arthritis, an autoinflammatory disease, presenting with frosted branch angiitis.
METHODS: A 14-year-old female with systemic juvenile idiopathic arthritis and a history of bilateral anterior uveitis developed acute unilateral vision loss and was found to have frosted branch angiitis complicated by branch retinal vein occlusion. She underwent an extensive serology workup and aqueous viral PCR to rule out other possible autoimmune and viral etiologies for forested branch angiitis. She received systemic and intravitreal antiviral treatment due to positive CMV IgM initially. However, the clinical picture improved following the use of a higher dose of oral steroids and the switch of the immunosuppressive agent to a TNF-a inhibitor.
CONCLUSIONS: To our knowledge, this would be the first case in the literature demonstrating a systemic juvenile idiopathic arthritis patient presenting with frosted branch angiitis. Infectious causes still must be ruled out, especially CMV, as it is the most common cause of secondary frosted branch angiitis.
METHODS: A 14-year-old female with systemic juvenile idiopathic arthritis and a history of bilateral anterior uveitis developed acute unilateral vision loss and was found to have frosted branch angiitis complicated by branch retinal vein occlusion. She underwent an extensive serology workup and aqueous viral PCR to rule out other possible autoimmune and viral etiologies for forested branch angiitis. She received systemic and intravitreal antiviral treatment due to positive CMV IgM initially. However, the clinical picture improved following the use of a higher dose of oral steroids and the switch of the immunosuppressive agent to a TNF-a inhibitor.
CONCLUSIONS: To our knowledge, this would be the first case in the literature demonstrating a systemic juvenile idiopathic arthritis patient presenting with frosted branch angiitis. Infectious causes still must be ruled out, especially CMV, as it is the most common cause of secondary frosted branch angiitis.
摘要：
背景：结霜分支血管炎是一种视网膜血管疾病，与病毒感染或自身免疫性疾病如克罗恩病有关，系统性红斑狼疮,和Behcet的病。结霜分支血管炎表现为血管炎症，视网膜水肿,和严重的视网膜血管鞘.我们介绍一例系统性幼年特发性关节炎,一种自身炎症性疾病,表现为磨砂分支血管炎。
方法：一名14岁女性，患有系统性幼年特发性关节炎，有双侧前葡萄膜炎病史，出现急性单侧视力丧失，发现结霜分支血管炎并发视网膜分支静脉阻塞。她接受了广泛的血清学检查和水性病毒PCR，以排除森林分支血管炎的其他可能的自身免疫和病毒病因。由于最初CMVIgM阳性，她接受了全身和玻璃体内抗病毒治疗。然而,在使用较高剂量的口服类固醇和将免疫抑制剂转换为TNF-α抑制剂后，临床表现有所改善。
结论：据我们所知，这将是文献中的首例病例,该病例证明系统性幼年特发性关节炎患者出现结霜分支血管炎.仍然必须排除传染病原因，尤其是CMV,因为它是继发性结霜分支血管炎的最常见原因。
方法：一名14岁女性，患有系统性幼年特发性关节炎，有双侧前葡萄膜炎病史，出现急性单侧视力丧失，发现结霜分支血管炎并发视网膜分支静脉阻塞。她接受了广泛的血清学检查和水性病毒PCR，以排除森林分支血管炎的其他可能的自身免疫和病毒病因。由于最初CMVIgM阳性，她接受了全身和玻璃体内抗病毒治疗。然而,在使用较高剂量的口服类固醇和将免疫抑制剂转换为TNF-α抑制剂后，临床表现有所改善。
结论：据我们所知，这将是文献中的首例病例,该病例证明系统性幼年特发性关节炎患者出现结霜分支血管炎.仍然必须排除传染病原因，尤其是CMV,因为它是继发性结霜分支血管炎的最常见原因。
