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孕期VKH的类固醇脉冲疗法:一种安全有效的选择?

Steroid pulse therapy for VKH during pregnancy: a safe and effective option?

作者信息

Sundararaju Umashri, Subramanian Shanmathi, Rajakumar Hamrish Kumar

机构信息

Government Medical College, Omandurar Government Estate, Chennai- 02, Tamilnadu, India.

出版信息

Orphanet J Rare Dis. 2025 Jul 16;20(1):366. doi: 10.1186/s13023-025-03916-9.

Abstract

Vogt-Koyanagi-Harada syndrome (VKH) is a rare autoimmune condition affecting melanocyte-containing tissues such as the eyes, skin, hair, inner ear, and meninges. The disease progresses through four phases: prodromal, uveitic, convalescent, and recurrent. Symptoms include headaches, photophobia, ocular inflammation, and auditory disturbances. Although the etiology behind VKH is unknown, it is believed to be triggered by viral infections such as the Epstein‒Barr virus or Cytomegalovirus in genetically predisposed individuals. It is diagnosed by confirming bilateral ocular involvement and ruling out other conditions, such as tuberculosis. It is managed by corticosteroids, with steroid pulse therapy being effective for controlling inflammation and improving outcomes. However, it carries significant risks, such as hypertension, infections, and electrolyte imbalances. Managing VKH during pregnancy can be challenging and requires a multidisciplinary approach, as corticosteroid use can affect both maternal and fetal health. Steroid therapy is crucial for controlling disease, but it can lead to complications such as infections, fetal growth restriction, and potential neurodevelopmental issues in infants. Future research should focus on optimizing steroid regimens, understanding the long-term effects of corticosteroid use during pregnancy, and developing personalized treatment strategies to improve maternal and fetal outcomes in VKH management.

摘要

伏格特-小柳-原田综合征(VKH)是一种罕见的自身免疫性疾病,会影响眼部、皮肤、毛发、内耳和脑膜等含黑素细胞的组织。该疾病会经历四个阶段:前驱期、葡萄膜炎期、恢复期和复发期。症状包括头痛、畏光、眼部炎症和听觉障碍。虽然VKH背后的病因尚不清楚,但据信在具有遗传易感性的个体中,它是由诸如爱泼斯坦-巴尔病毒或巨细胞病毒等病毒感染引发的。通过确认双侧眼部受累并排除其他疾病(如结核病)来进行诊断。它通过皮质类固醇进行治疗,类固醇脉冲疗法对于控制炎症和改善预后有效。然而,它存在重大风险,如高血压、感染和电解质失衡。在怀孕期间管理VKH具有挑战性,需要多学科方法,因为使用皮质类固醇会影响母婴健康。类固醇疗法对于控制疾病至关重要,但它可能导致感染、胎儿生长受限以及婴儿潜在的神经发育问题等并发症。未来的研究应专注于优化类固醇治疗方案,了解怀孕期间使用皮质类固醇的长期影响,并制定个性化治疗策略,以改善VKH管理中的母婴预后。

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