Divisions of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada.
Pediatr Rheumatol Online J. 2007 Dec 3;5:22. doi: 10.1186/1546-0096-5-22.
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.
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.
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.
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.
目前尚无敏感或特异性的实验室检测方法可用于测量局限性硬皮病(LS)的疾病活动度。监测几乎完全依赖于临床评估。我们的目的是确定血清 TGFβ1 浓度是否可作为儿童 LS 患者疾病活动度的良好生物标志物。
根据主要病变,将 55 例儿科 LS 患者分为硬斑病、弥漫性硬斑病、局限性硬斑病(影响四肢或面部)亚型;根据疾病活动度,对所有病变进行总体临床评估,分为活动期、非活动期和不确定期。采用酶联免疫吸附试验(ELISA)测量血清 TGFβ1 浓度水平,并进行分析,同时与疾病亚型和疾病活动度进行相关性分析。
患者组 TGFβ1 浓度平均值(51393±33953 pg/ml)明显高于对照组(9825±5287 pg/ml)(P<0.001)。所有疾病亚型的平均浓度均升高,且与疾病持续时间或活动度无关。
所有 LS 亚型患者的血清 TGFβ1 浓度升高,无论临床疾病活动度如何。尽管 TGFβ1 可能在局部皮肤纤维化的发病机制中起重要作用,但局部作用的已知分子的循环血液水平可能不是疾病活动度的有用生物标志物。