Research

Increased serum levels of TGFβ1 in children with localized scleroderma

Yosef Uziel^{* 1} , Brian M Feldman^{* 2,3} , Bernice R Krafchik^{* 4} , Ronald M Laxer^{* 2} and Rae SM Yeung^{* 2,5}

¹Meir Medical Center, Kfar Saba, Tel Aviv University, Israel

²Divisions of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada

³Departments of HPME and PHS, University of Toronto, Toronto, Canada

⁴Divisions Dermatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada

⁵Departments of Immunology and Medical Sciences, University of Toronto, Toronto, Canada

author email corresponding author email* Contributed equally

Pediatric Rheumatology 2007, 5:22doi:10.1186/1546-0096-5-22

Published:	3 December 2007

Abstract

Background

There are neither sensitive nor specific laboratory tests for measuring disease activity in localized scleroderma (LS). Monitoring is done almost exclusively by clinical assessment. Our aim was to determine whether serum concentrations of TGFβ1 are a good biomarker of disease activity in children with LS.

Methods

55 pediatric patients with LS were divided into sub-types according to their main lesion; morphea, generalized morphea, linear scleoderma affecting a limb or the face. The lesions were further categorized by overall clinical assessment into active, inactive, and indeterminate groups according to disease activity. Serum TGFβ1 concentration levels were measured by enzyme linked immunosorbent assay (ELISA), analyzed and correlated with disease subtypes and disease activity.

Results

The mean TGFβ1 concentration were significantly higher in the patient group (51393 ± 33953 pg/ml) than in the control group (9825 ± 5287 pg/ml) (P < 0.001). The mean concentration were elevated in all the disease subtypes, and did not correlate with disease duration or activity.

Conclusion

Serum concentration of TGFβ1 were elevated in patients with all subtypes of LS irrespective of clinical disease activity. Although TGFβ1 may play an important role in the pathogenesis of local skin fibrosis, circulating blood levels of molecules known to act locally may not be useful biomarkers of disease activity.