鸟性胆色素变异症与囊性黄斑水
Philippe Valmaggia1,2, Jordan Loeliger1, Oussama Habra1
1Eye Clinic, University Hospital Basel, Switzerland.
概括
鸟射胆色素病变 (BSCR) 的管理具有挑战性,特别是持续性囊性黄斑 (CME). 在一个具有挑战性的BSCR病例中,用mycophenolate mofetil和adalimumab联合治疗有效地减少了CME.
科学领域:
- 眼科医生 眼科 眼科
- 免疫学 免疫学 免疫学
背景情况:
- 鸟胆红蛋白病 (BSCR) 是一种罕见的双边炎症性眼病,与HLA-A29相关.
- 持续性囊性黄斑 (CME) 是一种常见的并发症,可以显著影响视觉预后.
研究的目的:
- 报告一个具有挑战性的BSCR病例与持久的CME.
- 评估顺序治疗方法在BSCR中管理耐火性CME的有效性.
主要方法:
- 基于SUN标准的BSCR诊断,包括 fundus发现和HLA-A29阳性.
- 通过实验室测试和成像,排除系统性炎症性疾病,如沙尔科毒症.
- 连续治疗全身性皮质类固醇和甲铁,其次是mycophenolate mofetil和adalimumab.
主要成果:
- 刚开始使用皮质类固醇和甲铁酸的治疗部分改善了视炎,但没有CME.
- 转换为mycophenolate mofetil和adalimumab导致CME显著减少,尽管一些残留的囊性变化仍然存在.
- 病程较晚的患者表现使CME管理复杂化.
结论:
- 在BSCR中囊性黄斑胀可能对初始治疗,包括全身性皮质类固醇不耐药.
- 与mycophenolate mofetil和adalimumab的联合治疗显示出在管理BSCR中持久性CME方面具有前途.
- 早期诊断,排除模仿者和灵活的成像指导治疗策略对于优化BSCR视觉结果至关重要.
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