Serum level of KL-6 as a marker of interstitial lung disease in patients with juvenile systemic sclerosis.
OBJECTIVE
Serum KL-6 has been found to be elevated in diseases characterized by diffuse interstitial lung involvement. The purpose of this study was to evaluate serum KL-6 as a marker of interstitial lung disease (ILD) in patients with juvenile systemic sclerosis (JSS).


METHODS
Serum concentrations of KL-6 were measured with an immunoassay in 39 serum samples from 12 children with diffuse cutaneous form of JSS (6 patients with and 6 patients without ILD) and from 20 healthy controls comparable for age. In patients sampled serially, the relationship of KL-6 concentrations with the severity of ILD and its response to treatment were evaluated.


RESULTS
Serum concentrations of KL-6 were significantly higher in patients with ILD (1687 +/- 979 IU/ml) than in patients without (345 +/- 95 IU/ml, p < 0.01) and healthy controls (311 +/- 114 IU/ml, p < 0.001). Serum KL-6 concentrations of patients without ILD were not statistically different from those of healthy controls. We found a significant correlation of serum KL-6 concentrations with vital capacity and with diffusing capacity for carbon monoxide (DLCO). Analysis of individual patients showed that serum concentrations of KL-6 were correlated with ILD severity and its response to treatment.


CONCLUSIONS
Measurement of serum KL-6 concentration is a useful noninvasive marker of pulmonary fibrosis in children with JSS. Its advantages over conventional methods of ILD assessment, such as pulmonary function test and high-resolution computerized tomography, are that it is easy to quantify and to measure repeatedly and it does not need children's cooperation.
Veselý
Richard
R
Klinika detí a_dorastu Fakultnej nemocnice s poliklinikou a Lekárskej fakulty UPJS, s.r.o. Kosice, Slovakia.
Vargová
Veronika
V
Ravelli
Angelo
A
Massa
Margherita
M
Oleksák
Eduard
E
D'Alterio
Rosanna
R
Martini
Alberto
A
De Benedetti
Fabrizio
F
D000293:Adolescent; D000941:Antigens / Q000097:blood*; D000951:Antigens, Neoplasm; D002648:Child; D002675:Child, Preschool; D005260:Female; D006023:Glycoproteins / Q000097:blood*; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000097:blood* / Q000150:complications / Q000503:physiopathology; D008297:Male; D018396:Mucin-1; D009077:Mucins; D012015:Reference Standards; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000097:blood* / Q000150:complications / Q000503:physiopathology
D000941:Antigens; D000951:Antigens, Neoplasm; D006023:Glycoproteins; C060192:MUC1 protein, human; D018396:Mucin-1; D009077:Mucins