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Pulmonary function tests in idiopathic inflammatory myopathy: association with clinical parameters in children.
- Source :
-
Arthritis care & research [Arthritis Care Res (Hoboken)] 2013 Sep; Vol. 65 (9), pp. 1424-31. - Publication Year :
- 2013
-
Abstract
- Objective: To determine the association of decreased lung function in children with idiopathic inflammatory myopathies (IIMs) with specific clinical parameters.<br />Methods: This study of 38 children ages 6-23 years diagnosed with definite/probable IIM evaluated the association of myositis-specific/-associated antibodies (MSAs/MAAs), duration of untreated disease at diagnosis, Disease Activity Score for muscle (DAS-M), muscle-derived enzymes (aldolase, lactate dehydrogenase [LDH], aspartate transaminase, and creatine phosphokinase [CPK]), neopterin and von Willebrand factor antigen, and the Childhood Myositis Assessment Scale (CMAS) scores with data from pulmonary function testing (PFT).<br />Results: Impaired PFTs were defined as total lung capacity (TLC) or diffusing capacity for carbon monoxide (DLCO) of <80% predicted. The PFTs documented that 37% of the children (14 of 38) had either decreased TLC or decreased DLCO; 5% (2 of 38) had both. Children with decreased TLC alone (7 [18%] of 38) were older both at the time of PFT and diagnosis, had anti-Jo-1 and anti-Scl-70 antibody, and had elevated levels of CPK and neopterin. Children with decreased DLCO alone (5 [13%] of 38) had a shorter duration of untreated disease at diagnosis, had higher DAS-M and total DAS, were positive for anti-Ro and anti-PL-12, had increased LDH, and had elevated levels of neopterin and aldolase, with low CMAS scores for items 1, 3, 10, 11, and 14.<br />Conclusion: Assessment of PFTs in children with IIMs should be considered, since more than one-third of patients were found to be impaired. The presence of MSAs/MAAs, an elevated serum neopterin level (mean ± SD 12.4 ± 9.6 nmoles/liter, normal value <10.5), older age at diagnosis, and shorter duration of untreated disease at diagnosis suggest the presence of potential lung pathology.<br /> (Copyright © 2013 by the American College of Rheumatology.)
Details
- Language :
- English
- ISSN :
- 2151-4658
- Volume :
- 65
- Issue :
- 9
- Database :
- MEDLINE
- Journal :
- Arthritis care & research
- Publication Type :
- Academic Journal
- Accession number :
- 23568855
- Full Text :
- https://doi.org/10.1002/acr.22014