Departamento de Reumatología Pediátrica, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City. Mexico.
Universidad Nacional Autónoma de México, Mexico City. Mexico.
Bol Med Hosp Infant Mex. 2021;78(5):385-394. doi: 10.24875/BMHIM.20000301.
Pulmonary involvement in juvenile systemic sclerosis (JSSc) is rare in children and contributes to morbimortality. This study aimed to describe the pulmonary function and clinical, radiologic, and tomographic findings in JSSc.
Patients with JSSc between 5-14 years of age were included. Clinical, functional, and imaging characteristics were assessed. Patients were excluded if they showed lung disease not associated with JSSc: mixed connective tissue disease, overlap syndrome, or acute cardiopulmonary failure at the time of the study. All patients underwent physical examination, electrocardiogram, spirometry, chest X-ray, high-resolution computed tomography (HRCT) of the chest, echocardiography, lung function tests, and the 6-minute walk test (6-MWT). Descriptive statistics were employed for data analysis.
We studied 15 patients with the following characteristics: median age, 11 years; median since symptoms onset, 6 years; median since JSSc diagnosis and the finding of pulmonary involvement, 2 years. Lung disease was detected in 73%, interstitial lung disease (ILD) the most common affection (67%); pulmonary hypertension was found in 6.6%. 6-MWT was positive in 26.6%, forced vital capacity (FVC) was abnormal in 26.6%. No pulmonary involvement was found in four patients.
The most frequent pulmonary affection in JSSc was ILD. Thus, early JSSc detection and periodic lung monitoring are mandatory to avoid further complications once JSSc is diagnosed.
儿童期发生的少年型系统性硬皮病（JSSc）肺部受累罕见，但与病死率相关。本研究旨在描述 JSSc 的肺功能和临床、放射学及断层扫描表现。
纳入年龄 5-14 岁的 JSSc 患者。评估其临床、功能和影像学特征。排除肺部疾病与 JSSc 无关的患者：混合性结缔组织病、重叠综合征或研究时急性心肺衰竭。所有患者均行体格检查、心电图、肺量测定、胸部 X 线、胸部高分辨率 CT（HRCT）、超声心动图、肺功能检查和 6 分钟步行试验（6-MWT）。数据分析采用描述性统计。
共纳入 15 例患者，具有以下特征：中位年龄 11 岁；症状起始中位时间 6 年；JSSc 诊断及肺部受累中位时间 2 年。73%的患者存在肺部疾病，最常见的肺部疾病为间质性肺疾病（ILD）（67%）；6.6%的患者存在肺动脉高压。26.6%的患者 6-MWT 阳性，26.6%的患者用力肺活量（FVC）异常。4 例患者无肺部受累。
JSSc 最常见的肺部病变为 ILD。因此，一旦诊断为 JSSc，必须早期发现并定期进行肺部监测，以避免进一步发生并发症。
