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Pulmonary function in childhood connective tissue diseases
I Cerveri1, C Bruschi, A Ravelli
1Institute of Respiratory Diseases, University of Pavia, Policlinico S. Matteo, Italy.
Insights
Pediatric patients with active connective tissue diseases (CTD) often show early lung function impairment, specifically reduced vital capacity (VC) and diffusing capacity of the lungs for carbon monoxide (DLCO), even without clinical signs.
Area of Science:
- Pediatric Rheumatology
- Pulmonary Medicine
- Immunology
Background:
- Connective tissue diseases (CTD) involve immune abnormalities and systemic inflammation.
- Pulmonary involvement is a known complication of CTD, but data in children are limited.
- Early detection of lung dysfunction in pediatric CTD is crucial.
Purpose of the Study:
- To investigate early lung function abnormalities in pediatric CTD patients.
- To assess the relationship between lung function and disease activity.
- To identify subclinical pulmonary involvement in children with CTD.
Main Methods:
- Studied 81 pediatric CTD patients without clinical/radiological pulmonary signs.
- Performed lung volume and diffusion lung capacity measurements.
- Compared results with 65 age/height-matched healthy controls.
Main Results:
- No significant differences in functional residual capacity (FRC) or maximal expiratory flow at 75% (MEF75) between groups.
- Significant impairment in vital capacity (VC) and diffusing capacity of the lungs for carbon monoxide (DLCO) in CTD patients.
- Impairment was more pronounced during the active phase of the disease.
Conclusions:
- Clinically active pediatric CTD is associated with functional lung impairment.
- VC and DLCO are sensitive indicators of subclinical lung involvement in pediatric CTD.
- Pulmonary function testing is recommended for active pediatric CTD cases, even without overt respiratory symptoms.
Abstract:
The term connective tissue diseases (CTD) defines a group of illnesses characterized by the presence of immune abnormalities and by widespread inflammation involving various organs and tissues including the lung. These diseases are not frequent in the paediatric age group. Very few data on pulmonary function are available in paediatric CTD. We investigated possible early lung function abnormalities and any likely relationship with clinical activity of the disease in a group of 81 paediatric CTD patients, without clinical or radiological evidence of pulmonary involvement. Measurement of lung volumes and diffusion lung capacity were performed. A sample of 65 subjects, defined as normal on the basis of history and clinical examination, and matched by age and height with the group of patients, was chosen as control group. CTD patients did not show significant deviations from the control distribution with respect to functional residual capacity (FRC) and maximal expiratory flow at 75% of the forced vital capacity (MEF75) values. On the contrary, both vital capacity (VC) and diffusing capacity of the lungs for carbon monoxide (DLCO) were quite impaired in most CTD during the active phase of the disease. Our results show a functional lung impairment in most children with clinically active CTD, even in absence of abnormalities on chest X-ray pictures.