Serum Level of KL-6 as a Marker of Interstitial Lung Disease in Patients with Juvenile Systemic Sclerosis

Richard Veselý, Veronika Vargová, Angelo Ravelli, Margherita Massa, Eduard Olekšák, Rosanna D'Alterio, Alberto Martini, Fabrizio De Benedetti

Research output: Contribution to journalArticlepeer-review


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 languageEnglish
Pages (from-to)795-800
Number of pages6
JournalJournal of Rheumatology
Issue number4
Publication statusPublished - Apr 2004


  • Interstitial lung disease
  • Juvenile systemic sclerosis
  • KL-6

ASJC Scopus subject areas

  • Rheumatology
  • Immunology


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