%0 Journal Article
%T Increased serum levels of TGF¦Â1 in children with localized scleroderma
%A Yosef Uziel
%A Brian M Feldman
%A Bernice R Krafchik
%A Ronald M Laxer
%A Rae SM Yeung
%J Pediatric Rheumatology
%D 2007
%I BioMed Central
%R 10.1186/1546-0096-5-22
%X 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 TGF¦Â1 concentration levels were measured by enzyme linked immunosorbent assay (ELISA), analyzed and correlated with disease subtypes and disease activity.The mean TGF¦Â1 concentration were significantly higher in the patient group (51393 ¡À 33953 pg/ml) than in the control group (9825 ¡À 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.Serum concentration of TGF¦Â1 were elevated in patients with all subtypes of LS irrespective of clinical disease activity. Although TGF¦Â1 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.Localized scleroderma (LS) is the most common type of scleroderma in children. LS differs from systemic sclerosis (SSc) by typically being confined to the skin and subcutaneous tissue, with only rare involvement of the internal organs [1,2]. 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.The pathogenesis of the skin fibrosis is far from clear; like other autoimmune diseases it is thought to involve an environmental trigger in an immuno-susceptible host leading to inflammation and damage, with increased production and deposition of collagen [3]. The Transforming Growth Factor ¦Â family of cytokines (TGF¦Â) plays a major role in modulation of the immune response, and is a critical counter
%U http://www.ped-rheum.com/content/5/1/22