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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.

The European Respiratory Journal

|June 1, 1992

Summary

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.

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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.

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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.