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Updated: Oct 19, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary involvement in patients with juvenile systemic sclerosis
Adriana I Céspedes-Cruz1,2, Rosa A Carranza-Muleiro3,4, Elva L López-Rojas5
1Departamento de Reumatología Pediátrica, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social (IMSS), Mexico City. Mexico.
Insights
Juvenile systemic sclerosis (JSSc) can affect the lungs, with interstitial lung disease (ILD) being the most common finding. Early detection and monitoring of pulmonary function in children with JSSc are crucial for managing this rare condition.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Medical Imaging
Background:
- Pulmonary involvement is a rare but significant complication in juvenile systemic sclerosis (JSSc), impacting morbidity and mortality in children.
- Understanding the spectrum of lung disease in JSSc is crucial for timely diagnosis and management.
Purpose of the Study:
- To characterize pulmonary function, clinical, radiological, and tomographic findings in children diagnosed with JSSc.
- To identify the prevalence and types of lung involvement in a cohort of pediatric patients with JSSc.
Main Methods:
- A cohort of 15 patients aged 5-14 years with JSSc and pulmonary involvement was studied.
- Comprehensive assessments included physical examination, spirometry, chest X-ray, high-resolution computed tomography (HRCT), echocardiography, lung function tests, and the 6-minute walk test (6-MWT).
- Exclusion criteria targeted unrelated lung diseases and acute cardiopulmonary failure.
Main Results:
- Pulmonary involvement was identified in 73% of the studied JSSc patients.
- Interstitial lung disease (ILD) was the most common pulmonary manifestation, present in 67% of affected patients.
- Abnormalities in the 6-minute walk test (6-MWT) and forced vital capacity (FVC) were observed in 26.6% of patients; pulmonary hypertension was found in 6.6%.
Conclusions:
- Interstitial lung disease (ILD) is the predominant pulmonary complication in juvenile systemic sclerosis (JSSc).
- Routine screening for pulmonary involvement, including lung function tests and imaging, is essential following JSSc diagnosis.
- Early detection and consistent monitoring are critical to prevent severe complications associated with JSSc-related lung disease.
Background:
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.
Methods:
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.
Results:
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.
Conclusions:
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.
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