Machado Daniela, Lima Filipa, Marques Catarina, Monteiro Regina
Pulmonology Department, Gaia/Espinho Hospital Center, Vila Nova de Gaia, Portugal.
Medicine (Baltimore). 2019 Feb;98(6):e14269. doi: 10.1097/MD.0000000000014269.
Swyer-James-Macleod syndrome (SJMS) is a rare lung disorder characterized by unilateral hyperlucent lungs which arises as a complication of bronchiolitis obliterans. It is typically diagnosed during childhood, although some patients may only be diagnosed in adulthood, often as an incidental finding, but others due to recurrent chest infections.
Three patients were referred to our institution with complaints of dyspnea on exertion, chronic productive cough, and recurrent pulmonary infections. Two of them had a history of lower respiratory tract infections during childhood.
A computed tomography scan was performed and showed unilateral reduced density of the lung and bronchiectasis in the 3 patients. Based on the clinical presentation and radiologic features, the diagnosis of SJMS was established.
Patients started inhaled corticosteroids and long acting beta agonist, vaccines to prevent respiratory infections were administered and airway clearance techniques were taught.
Two patients became asymptomatic with inhaled therapy and no recurrent chest infections were observed over a 3-year follow-up period, being discharged from our institution to the general practitioner. The 3rd patient had some improvement in the frequency of pulmonary infections and dyspnea, without improvement in respiratory function tests, maintaining vigilance at our center.
These cases highlight the importance of being aware of this condition and its frequent association with bronchiectasis, which may adversely affect the prognosis, to manage patients appropriately and prevent recurrent pulmonary infections.
斯怀尔-詹姆斯-麦克劳德综合征（SJMS）是一种罕见的肺部疾病，其特征为单侧肺透亮度增加，是闭塞性细支气管炎的一种并发症。该病通常在儿童期被诊断出来，不过有些患者可能直到成年才被诊断，常常是偶然发现，但也有一些是因为反复出现胸部感染。
三名患者因活动时呼吸困难、慢性咳痰性咳嗽和反复肺部感染的主诉被转诊至我院。其中两名患者有儿童期下呼吸道感染病史。
进行了计算机断层扫描，结果显示3名患者单侧肺密度降低且有支气管扩张。根据临床表现和影像学特征，确诊为SJMS。
患者开始吸入皮质类固醇和长效β受体激动剂，接种预防呼吸道感染的疫苗，并教授气道清理技术。
两名患者通过吸入治疗后无症状，在3年随访期内未观察到反复胸部感染，从我院出院后转至全科医生处。第三名患者肺部感染频率和呼吸困难有所改善，但呼吸功能测试无改善，仍在我院接受密切观察。
这些病例凸显了认识这种疾病及其与支气管扩张的常见关联的重要性，支气管扩张可能对预后产生不利影响，以便对患者进行适当管理并预防反复肺部感染。
