Serum KL-6 level as a biomarker of interstitial lung disease in childhood connective tissue diseases: a pilot study
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Rheumatology International https://doi.org/10.1007/s00296-019-04485-4
INTERNATIONAL
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Serum KL‑6 level as a biomarker of interstitial lung disease in childhood connective tissue diseases: a pilot study Ayse Ayzit Kilinc1 · Asli Arslan1 · Mehmet Yildiz2 · Mine Kucur3 · Amra Adrovic2 · Kenan Barut2 · Sezgin Sahin2 · Haluk Cokugras1 · Ozgur Kasapcopur2 Received: 11 September 2019 / Accepted: 23 November 2019 © Springer-Verlag GmbH Germany, part of Springer Nature 2019
Abstract Krebs von den Lungen-6 (KL-6) has been described as a promising biomarker in the diagnosis and determining the severity of interstitial lung disease in adults with connective tissue disease. This study was performed to determine whether the serum KL-6 level is useful as a biomarker for detecting the interstitial lung disease (ILD) in pediatric cases of connective tissue disease (CTD). In total, 88 patients [36 patients with systemic juvenile idiopathic arthritis (sJIA), 27 patients with juvenile systemic lupus erythematosus (JSLE), 14 patients with juvenile dermatomyositis (JDM), and 11 patients with juvenile systemic sclerosis (JSSc)] and 68 healthy controls were included in this study. Age, sex, and duration of CTD and ILD (if any) were recorded. Blood samples from all the patients and controls were examined by ELISA. Eleven of the 88 patients with CTD (12.5%) had ILD and all of them were symptomatic. Subgroup analysis indicated that eight patients had JSSc, two had JSLE, and one had systemic JIA. The median serum KL-6 level was 1450.5 U/mL (interquartile range (IQR) 742.9–2603.2) in the CTD with ILD group, 415.9 U/mL (IQR 233.4–748.4) in the CTD without ILD group, and 465.9 U/ mL (IQR 273.6–1036) in the control group. KL-6 levels were significantly higher in the CTD with ILD group than the CTD without ILD group and the control group (p = 0.003). At a cut-off of 712.5 U/ml identified by ROC curve, serum KL-6 yielded a sensitivity of 81% and specificity of 72% for CTD with ILD group. There was no significant difference in serum KL-6 level among the disease subgroups (sJIA, JSLE, JSSc, JDM), in either the CTD with ILD group or the CTD without ILD group (p > 0.05). In conclusion, KL-6 is a useful biomarker of CTD with ILD in pediatric patients. Keywords Interstitial lung diseases · Connective tissue diseases · Systemic sclerosis · Juvenile idiopathic arthritis · Systemic lupus erythematosus · KL-6
Introduction Systemic juvenile idiopathic arthritis (sJIA), juvenile systemic lupus erythematosus (JSLE), juvenile systemic sclerosis (JSSc), and juvenile dermatomyositis (JDM) are * Ozgur Kasapcopur [email protected] 1
Department of Pediatric Pulmonology, Cerrahpasa Faculty of Medicine, İstanbul University-Cerrahpasa, İstanbul, Turkey
2
Department of Pediatric Rheumatology, Cerrahpasa Faculty of Medicine, İstanbul University-Cerrahpasa, İstanbul, Turkey
3
Department of Biochemistry, Cerrahpasa Faculty of Medicine, İstanbul University-Cerrahpasa, İstanbul, Turkey
immune-mediated connective tissue di
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