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Abnormalities in lung volumes and airflow in children with newly diagnosed connective tissue disease
Joanna Peradzyńska, Katarzyna Krenke1, Anna Szylling
1Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw. katarzynakrenke@gmail.com.
Insights
Pulmonary function abnormalities are common in children with childhood connective tissue diseases (CTDs). Initial assessment revealed airflow limitation in 42% of pediatric CTD patients, with no progression over two years.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Internal Medicine
Background:
- Childhood connective tissue diseases (CTDs) are rare inflammatory disorders affecting multiple organs.
- Pulmonary involvement in pediatric CTDs is uncommon but can lead to significant functional impairment.
- Limited data exists on the prevalence and types of lung function abnormalities in children with CTDs.
Purpose of the Study:
- To assess the pulmonary functional status in children newly diagnosed with CTD.
- To monitor changes in lung function over a two-year disease course.
Main Methods:
- A cohort of 98 children with newly diagnosed CTD and 80 healthy controls were enrolled.
- Methods included medical history, physical examination, chest radiography, and pulmonary function testing (PFT) via spirometry and whole-body plethysmography.
- PFT was reassessed after 24 months.
Main Results:
- Children with CTDs showed significantly lower FEV₁, FEV₁/FVC, and MEF50 compared to controls.
- 42% of CTD patients exhibited abnormal lung function, versus 11% in the control group.
- No significant progression of lung function impairment was observed over the 24-month follow-up period.
Conclusions:
- Lung function impairment is a frequent finding in children diagnosed with CTDs.
- While restrictive patterns are typical, airflow limitation can be an initial indicator of lung involvement in pediatric CTDs.
Introduction:
Connective tissue diseases (CTDs) of childhood are rare inflammatory disorders, involving various organs and tissues including respiratory system. Pulmonary involvement in patients with CTDs is uncommon but may cause functional impairment. Data on prevalence and type of lung function abnormalities in children with CTDs are scarce. Thus, the aim of this study was to asses pulmonary functional status in children with newly diagnosed CTD and follow the results after two years of the disease course.
Material And Methods:
There were 98 children (mean age: 13 ± 3; 76 girls), treated in Department of Pediatric Rheumatology, Institute of Rheumatology, Warsaw and 80 aged-matched, healthy controls (mean age 12.7 ± 2.4; 50 girls) included into the study. Study procedures included medical history, physical examination, chest radiograph and PFT (spirometry and whole body-plethysmography). Then, the assessment of PFT was performed after 24 months.
Results:
FEV₁, FEV₁/FVC and MEF50 were significantly lower in CTD as compared to control group, there was no difference in FVC and TLC. The proportion of patients with abnormal lung function was significantly higher in the study group, 41 (42%) vs 9 (11%). 24-months observation didn't reveal progression in lung function impairment.
Conclusions:
Lung function impairment is relatively common in children with CTDs. Although restrictive ventilatory pattern is considered typical feature of lung involvement in CTDs, airflow limitation could also be an initial abnormality.
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