Abstract
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. Conclusion. 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.
| Original language | English |
|---|---|
| Pages (from-to) | 795-800 |
| Number of pages | 6 |
| Journal | Journal of Rheumatology |
| Volume | 31 |
| Issue number | 4 |
| Publication status | Published - Apr 2004 |
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Keywords
- Interstitial lung disease
- Juvenile systemic sclerosis
- KL-6
ASJC Scopus subject areas
- Rheumatology
- Immunology
Cite this
Serum Level of KL-6 as a Marker of Interstitial Lung Disease in Patients with Juvenile Systemic Sclerosis. / Veselý, Richard; Vargová, Veronika; Ravelli, Angelo; Massa, Margherita; Olekšák, Eduard; D'Alterio, Rosanna; Martini, Alberto; De Benedetti, Fabrizio.
In: Journal of Rheumatology, Vol. 31, No. 4, 04.2004, p. 795-800.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Serum Level of KL-6 as a Marker of Interstitial Lung Disease in Patients with Juvenile Systemic Sclerosis
AU - Veselý, Richard
AU - Vargová, Veronika
AU - Ravelli, Angelo
AU - Massa, Margherita
AU - Olekšák, Eduard
AU - D'Alterio, Rosanna
AU - Martini, Alberto
AU - De Benedetti, Fabrizio
PY - 2004/4
Y1 - 2004/4
N2 - 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. Conclusion. 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.
AB - 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. Conclusion. 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.
KW - Interstitial lung disease
KW - Juvenile systemic sclerosis
KW - KL-6
UR - http://www.scopus.com/inward/record.url?scp=1842425819&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=1842425819&partnerID=8YFLogxK
M3 - Article
C2 - 15088311
AN - SCOPUS:1842425819
VL - 31
SP - 795
EP - 800
JO - Journal of Rheumatology
JF - Journal of Rheumatology
SN - 0315-162X
IS - 4
ER -