Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey; and.
Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Pediatrics. 2019 Aug;144(2). doi: 10.1542/peds.2019-0162. Epub 2019 Jul 18.
Behçet 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-α2a 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.
贝赫切特病（BD）是一种多系统自身炎症性疾病，其特征为反复发作的黏膜皮肤、眼部、肌肉骨骼、胃肠道、中枢神经系统和血管表现。BD 的肺血管受累（PAI）可能是血管炎最严重的形式，至少在儿童中是这样。PAI 的死亡率、发病率和复发率都很高。关于伴有 PAI 的儿童 BD 患者的治疗和结局的数据有限。在此，我们报告了 2 例以咯血为表现的 BD 患儿，并通过系统回顾支持我们的数据。这些患者接受了免疫抑制治疗，包括脉冲甲基泼尼松龙，随后口服泼尼松龙，每 3 周静脉注射环磷酰胺，共 6 个周期，同时使用干扰素-α2a。这是文献中首次报道的成功采用这种治疗方式治疗并发症（死亡率为 50%）的病例。这些患者的随访时间至少为 4 年，没有任何血管复发。儿科医生应该意识到，BD 患者可能不符合全部诊断标准。对于有 PAI 的儿童，他们应考虑到 BD 的可能性，以避免诊断延误，并开始进行挽救生命的准确免疫抑制治疗。
