Abstract
BackgroundThere 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.Methods55 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.ResultsThe 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.ConclusionSerum 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.
Highlights
There are neither sensitive nor specific laboratory tests for measuring disease activity in localized scleroderma (LS)
The patients were divided into four clinical subtypes according to their main lesion: Morphea (M), generalized morphea (GM) when they had a large area of the body involved and confluent or multiple morpheic lesions (3 or more lesions), or a linear band with 2 or more lesions, and linear scleroderma (LIN) on a limb (LIN limb) or on the face (LIN face "en coup de sabre")
The mean serum TGFβ1 concentrations were significantly higher in patients than controls: 51393 ± 33953 pg/ml, vs. 9825 ± 5287 pg/ml
Summary
There are neither sensitive nor specific laboratory tests for measuring disease activity in localized scleroderma (LS). Our aim was to determine whether serum concentrations of TGFβ1 are a good biomarker of disease activity in children with LS. There are neither sensitive nor specific laboratory tests for measuring disease activity, and monitoring is done almost exclusively by clinical assessment, which is often challenging. Identifying a laboratory marker of disease activity will aid in the management of affected children. Several studies have found elevated serum TGFβ1 concentrations in both generalized and localized forms of sclerosis [4,5,6]. Our study objectives were to determine the peripheral blood levels of TGFβ1 in children with LS and to determine the relationship of serum levels to disease activity
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