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Increased serum levels of TGFbeta1 in children with localized scleroderma
Yosef Uziel1, Brian M Feldman, Bernice R Krafchik
1Divisions of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada. rae.yeung@sickkids.CA.
Insights
Serum levels of transforming growth factor beta 1 (TGFbeta1) are elevated in children with localized scleroderma (LS). However, TGFbeta1 is not a reliable biomarker for measuring LS disease activity.
Area of Science:
- Dermatology
- Pediatric Rheumatology
- Immunology
Background:
- Localized scleroderma (LS) lacks sensitive and specific laboratory tests for disease activity monitoring.
- Current LS monitoring relies heavily on clinical assessment.
- The study investigated serum TGFbeta1 as a potential biomarker for LS activity in children.
Purpose of the Study:
- To evaluate serum concentrations of transforming growth factor beta 1 (TGFbeta1) as a biomarker for disease activity in pediatric localized scleroderma (LS).
Main Methods:
- Fifty-five pediatric LS patients were classified by lesion subtype (morphea, generalized morphea, linear scleroderma) and disease activity (active, inactive, indeterminate).
- Serum TGFbeta1 levels were quantified using enzyme-linked immunosorbent assay (ELISA).
- TGFbeta1 concentrations were analyzed and correlated with LS subtypes and disease activity.
Main Results:
- Mean serum TGFbeta1 concentration was significantly higher in LS patients (51393 pg/ml) compared to controls (9825 pg/ml) (P < 0.001).
- Elevated TGFbeta1 levels were observed across all LS subtypes.
- TGFbeta1 concentrations did not correlate with disease duration or clinical activity.
Conclusions:
- Serum TGFbeta1 is elevated in all localized scleroderma subtypes, regardless of clinical disease activity.
- While TGFbeta1 is implicated in skin fibrosis pathogenesis, its circulating levels may not serve as effective biomarkers for LS disease activity.
- Further research is needed to identify reliable biomarkers for localized scleroderma activity.
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 TGFbeta1 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 TGFbeta1 concentration levels were measured by enzyme linked immunosorbent assay (ELISA), analyzed and correlated with disease subtypes and disease activity.
Results:
The mean TGFbeta1 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 TGFbeta1 were elevated in patients with all subtypes of LS irrespective of clinical disease activity. Although TGFbeta1 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.
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