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Updated: Mar 29, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
The frequency of pulmonary hypertension in patients with juvenile scleroderma
Amra Adrovic1, Funda Oztunc, Kenan Barut
1Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. aadrovic@yahoo.com.
Insights
Juvenile scleroderma (JS) rarely involves the heart or lungs. Non-invasive tests like Doppler echocardiography and pulmonary function tests showed normal cardiopulmonary parameters in 35 JS patients, indicating uncommon cardiac and pulmonary involvement.
Area of Science:
- Pediatric Rheumatology
- Cardiopulmonary Medicine
- Connective Tissue Diseases
Background:
- Juvenile scleroderma (JS) is a rare connective tissue disease with potential for multi-organ involvement.
- Cardiac and pulmonary complications in JS can significantly increase mortality and morbidity in affected children.
- Early detection of cardiopulmonary involvement is crucial for managing JS patients.
Purpose of the Study:
- To evaluate non-invasive methods for assessing cardiopulmonary involvement in juvenile scleroderma.
- To investigate Doppler echocardiography and pulmonary function tests (FVC, DLCO) in JS patients.
- To determine the prevalence of cardiopulmonary complications in a cohort of JS patients.
Main Methods:
- Thirty-five patients with confirmed JS were assessed.
- Doppler echocardiography was used to measure parameters like tricuspid insufficiency (TI), pulmonary insufficiency (PI), and acceleration time/ejection time (AT/ET) ratio.
- Pulmonary function was evaluated using Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO).
Main Results:
- All 35 patients with juvenile scleroderma exhibited normal values for all tested cardiopulmonary parameters.
- Specifically, TI, PI, AT/ET ratio, pulmonary arterial pressure (PAP), FVC, and DLCO were within normal ranges.
- This suggests a low incidence of cardiopulmonary involvement in the studied JS cohort.
Conclusions:
- Non-invasive assessments, including Doppler echocardiography and pulmonary function tests, revealed no significant cardiopulmonary involvement in the studied group of juvenile scleroderma patients.
- The findings indicate that cardiopulmonary complications are uncommon in this population.
- Further research may be warranted to confirm these findings in larger cohorts.
Abstract:
Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.
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