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Serum Level of KL-6 as a Marker of Interstitial Lung Disease in Patients with Juvenile Systemic Sclerosis
RICHARD VESELY, VERONIKA VARGOVÁ, ANGELO RAVELLI, MARGHERITA MASSA, EDUARD OLEKSÁK, ROSANNA D'ALTERIO, ALBERTO MARTINI, and FABRIZIO DE BENEDETTI
ABSTRACT.
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. (J Rheumatol 2004;31:795-800) Key Indexing Terms:
KL-6
From the Klinika detí a_dorastu Fakultnej nemocnice s poliklinikou a Lekárskej fakulty UPJS and the MEDY, s.r.o. Kosice, Slovakia; the Dipartimento di Pediatria, Università di Genova, Unità Operativa Pediatria II, IRCCS G. Gaslini, Genova; the Laboratorio di Biotecnologie e Tecnologie Biomediche, IRCCS Policlinico San Matteo and the Dipartimento di Pediatria, Università di Pavia, IRCCS Policlinico San Matteo, Pavia; and the Laboratorio di Reumatologia, Pediatria 2 Reumatologia, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy. R. Vesely, MD, Pediatric Rheumatologist; V. Vargová, MD, PhD, Assistant Professor of Pediatrics, Klinika detí a dorastu Fakultnej nemocnice s poliklinikou a Lekárskej fakulty UPJS; A. Ravelli, MD, PhD; F. De Benedetti, MD, PhD, Dirigente Medico di I Livello; A. Martini, MD, Professor of Pediatrics, Dipartimento di Pediatria, Università di Genova; M. Massa, PhD, PhD, Dirigente Biologo di I Livello, Laboratorio di Biotecnologie e Tecnologie Biomediche; E. Oleksák, MD, Director of Clinical Laboratory, MEDY, s.r.o.; R. D'Alterio, MD, Resident, Dipartimento di Pediatria, Università di Pavia. Address reprint requests to Dr. R. Vesely, Klinika detí a dorastu Fakultnej nemocnice s poliklinikou a Lekárskej fakulty UPJS, Kosice, Slovakia. Submitted February 13, 2003; revision accepted October 15, 2003. |