Pediatric Rheumatology Online Journal (Dec 2007)

Increased serum levels of TGFβ1 in children with localized scleroderma

  • Krafchik Bernice R,
  • Feldman Brian M,
  • Uziel Yosef,
  • Laxer Ronald M,
  • Yeung Rae SM

DOI
https://doi.org/10.1186/1546-0096-5-22
Journal volume & issue
Vol. 5, no. 1
p. 22

Abstract

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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 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.