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Pulmonary function in children affected by juvenile spondyloarthropathy
G Camiciottoli1, S Trapani, M Ermini
1Department of Clinical Pathophysiology, University of Florence, Italy.
Insights
Pulmonary function tests revealed that 33% of juvenile spondyloarthropathy (JSpA) patients had lung impairment, primarily a restrictive pattern. No changes or correlations with disease factors were observed over two years.
Area of Science:
- Pediatric Rheumatology
- Pulmonary Medicine
- Clinical Investigation
Background:
- Pleuropulmonary involvement is recognized in adult spondyloarthropathy (SpA), often presenting as upper lobe fibrosis on radiology.
- Pulmonary function tests (PFTs) in adults with SpA typically show reduced vital capacity and total lung capacity.
- Pulmonary involvement in juvenile SpA (JSpA) is understudied, with limited data on PFT alterations.
Purpose of the Study:
- To investigate the prevalence and characteristics of PFT alterations in JSpA patients over a two-year follow-up.
- To analyze the relationship between PFT findings and disease duration, activity, and chest/spine mobility in JSpA.
Main Methods:
- Eighteen JSpA patients without clinical lung disease or abnormal chest radiographs underwent PFTs (volumes, diffusing capacity for carbon monoxide [DLCO]) at baseline, 12, and 24 months.
- Disease activity was assessed using clinical and hematological data at each time point.
Main Results:
- Significant PFT impairment was found in 33% of patients, including reduced forced vital capacity (22%) and DLCO (11%).
- No significant changes in PFT alterations were observed between 12 and 24 months.
- No correlation was identified between PFT results and disease duration, activity, or clinical scores.
Conclusions:
- A substantial proportion (33%) of JSpA patients exhibit PFT alterations, predominantly a restrictive pattern, despite the absence of clinical or radiological lung findings.
- PFT abnormalities did not progress or change over the two-year study period.
- No association was found between PFT alterations and disease parameters like duration or activity in this cohort.
Objective:
Pleuropulmonary involvement in adult spondyloarthropathy (SpA) has been thoroughly investigated. SpA is usually detected by conventional radiology as fibrosis of the upper lobes in about 30% of asymptomatic patients. Pulmonary function tests (PFT) reveal decreased vital capacity and total lung capacity, as well as increased residual volume. Juvenile SpA (JSpA) is a rare clinical condition, and no extensive investigations have been carried out on pulmonary involvement in JSpA. We studied prevalence and features of PFT alterations in patients with JSpA over a 2 year followup and analyzed the relationship between PFT and disease duration, disease activity, and chest and spine mobility.
Methods:
Eighteen patients with JSpA, with no clinical signs of lung disease and normal chest radiographs, underwent PFT--static and dynamic volumes, diffusing capacity for carbon monoxide (DLCO), at enrollment (T0), at 12 months (T1), and at 24 months later (T2). Disease activity was defined at each investigation by clinical and hematological data.
Results:
Significant functional lung impairment was detected in 33% of patients (reduced forced vital capacity in 22% and DLCO in 11%). No significant change in the prevalence and features of PFT alterations was detected at T1 and T2; no relationship was found between PFT and duration, activity, and clinical scores of the disease.
Conclusion:
Thirty-three percent of JSpA patients without clinical symptoms and no radiological findings of lung involvement show PFT alterations, mainly characterized by a restrictive pattern. No progression or modification in PFT developed over 2 years. No correlation was found between PFT and disease duration, activity, and clinical scores.
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