Pulmonary involvement in patients with juvenile systemic sclerosis

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.