Abstract
BackgroundIn adults with rheumatic diseases pulmonary complications are relevant contributors to morbidity and mortality. In these patients diffusion capacity for CO (DLCO) is an established method to detect early pulmonary impairment. Pilot studies using DLCO indicate that early functional pulmonary impairment is present even in children with rheumatic disease albeit not detectable by spirometry and without clinical signs of pulmonary disease.Since the lung clearance index (LCI) is also a non-invasive, feasible and established method to detect early functional pulmonary impairment especially in children and because it requires less cooperation (tidal breathing), we compared LCI versus DLCO (forced breathing and breath-holding manoeuvre) in children with rheumatic diseases.FindingsNineteen patients (age 9–17 years) with rheumatic disease and no clinical signs of pulmonary disease successfully completed LCI and DLCO during annual check-up. In 2 patients LCI and DLCO were within physiological limits. By contrast, elevated LCI combined with physiological results for DLCO were seen in 8 patients and in 9 patients both, the LCI and DLCO indicate early functional pulmonary changes. Overall, LCI was more sensitive than DLCO to detect early functional pulmonary impairment (p = 0.0128).ConclusionsOur findings suggest that early functional pulmonary impairment is already present in children with rheumatic diseases. LCI is a very feasible and non-invasive alternative for detection of early functional pulmonary impairment in children. It is more sensitive and less cooperation dependent than DLCO. Therefore, we suggest to integrate LCI in routine follow-up of rheumatic diseases in children.
Highlights
Our findings suggest that early functional pulmonary impairment is already present in children with rheumatic diseases
Adults with rheumatic diseases are at risk to develop pulmonary impairment secondary to the disease-related chronic inflammation and fibrosis, and/or to a lesser extent due to immunosuppressive therapy
Two recently published studies on children and young adults with juvenile idiopathic arthritis (JIA) with no clinical signs of pulmonary disease and healthy controls stated a significant reduction in Diffusion capacity for CO (DLCO) in JIA patients, while spirometry data were fairly comparable between groups
Summary
Adults with rheumatic diseases are at risk to develop pulmonary impairment secondary to the disease-related chronic inflammation and fibrosis, and/or to a lesser extent due to immunosuppressive therapy. During follow-up the DLCO (CO specific diffusion capacity of the lung) has been shown to be a more sensitive marker for early pulmonary impairment than spirometry [1] By contrast, this issue has rarely been investigated in pediatric patients with rheumatic diseases, most probably due to the fact that breathing manoeuvre required for DLCO (forced breathing and breath-holding for a mandatory length of time) are challenging. Two recently published studies on children and young adults with juvenile idiopathic arthritis (JIA) with no clinical signs of pulmonary disease and healthy controls stated a significant reduction in DLCO in JIA patients, while spirometry data were fairly comparable between groups This data indicates, that even pediatric JIA patients are prone to early functional pulmonary impairment not detectable by spirometry [2, 3]. Since the lung clearance index (LCI) is a non-invasive, feasible and established method to detect early functional pulmonary impairment especially in children and because it requires less cooperation (tidal breathing), we compared LCI versus DLCO (forced breathing and breath-holding manoeuvre) in children with rheumatic diseases
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