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首页> 外文期刊>Pediatrics: Official Publication of the American Academy of Pediatrics >The Challenge of Treating Pulmonary Vasculitis in Behcet Disease: Two Pediatric Cases
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The Challenge of Treating Pulmonary Vasculitis in Behcet Disease: Two Pediatric Cases

机译：在Behcet疾病中治疗肺血管炎的挑战：两位儿科病例

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摘要

Behcet disease (BD) is a multisystemic autoinflammatory disorder characterized by recurrent mucocutaneous, ocular, musculoskeletal, gastrointestinal, central nervous system, and vascular manifestations. Pulmonary arterial involvement (PAI) of BD is probably the most severe form of vasculitis, at least in children. PAI has a high mortality, morbidity, and recurrence rate. There are limited data regarding treatment and outcomes of pediatric patients with BD with PAI. Herein, we report 2 pediatric patients with BD presented with hemoptysis and support our data with a systematic review. These patients were given immunosuppressive therapy, which covered pulse methylprednisolone followed by oral prednisolone, intravenous cyclophosphamide every 3 weeks for a total of 6 cycles, and interferon-alpha 2a concomitantly. These are the first reported cases in the literature successfully treated with this treatment modality in a complication with 50% mortality. These patients have been followed up for a period of at least 4 years without any vascular recurrence. Pediatricians should be aware that patients with BD may not present with full diagnostic criteria. They should consider BD in a child with PAI to avoid diagnostic delay and start life-saving accurate immunosuppressive treatment.
机译：Behcet疾病（BD）是一种多系统的自身炎症障碍，其特征是经复制的粘膜皮肤，眼镜，肌肉骨骼，胃肠道，中枢神经系统和血管表现形式。 BD的肺动脉受累（PAI）可能是最严重的血管炎，至少在儿童中。 PAI具有高死亡率，发病率和复发率。有关PAI的BD患者的治疗和结果存在有限的数据。在此，我们报告了2名儿科患者患有咯血的BD，并通过系统审查支持我们的数据。将这些患者提供免疫抑制治疗，其覆盖脉冲甲基己酮，然后口服泼尼松龙，每3周静脉环磷酰胺，总共6个循环，以及干扰素-α2a。这些是文献中的第一个报告的病例，在该治疗方式中成功处理了50％死亡率的并发症。这些患者在没有任何血管复发的情况下进行了至少4年的时间。儿科医生应该意识到BD的患者可能无法满足完全诊断标准。他们应该考虑一个带有PAI的孩子的BD，以避免诊断延迟并开始救生准确的免疫抑制治疗。

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