Massabki Paulo S, Silva Neusa P, Lourenço Dayse M, Andrade Luis E C
Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo (UNIFESP), Rua Botucatu 740, São Paulo, SP 04023-062, Brazil.
J Rheumatol. 2003 Dec;30(12):2606-12.
To determine frequency, origin, and clinical associations of elevated serum neuron specific enolase (NSE) in systemic sclerosis (SSc).
Serum was obtained from 75 patients with SSc, 20 systemic lupus erythematosus, 8 polymyositis, 10 idiopathic interstitial lung disease, and 10 healthy volunteers. NSE status was determined in serum (in all individuals) and in platelet lysate (in volunteers and 30 patients with SSc).
Elevated serum NSE (mean 22.6 ng/ml, range 12.1-68.2 ng/ml) was observed in 26 patients with SSc (34.6%). Those with diffuse SSc had higher serum NSE than those with limited disease (16.5 +/- 13.4 vs 9.6 +/- 5.0 ng/ml, p = 0.006). No association was found between serum NSE and lung or esophagus involvement. Patients with long-standing disease had lower serum NSE than those with early disease (10.8 +/- 7.3 vs 16.1 +/- 13.6 ng/ml, p = 0.05). Serum NSE was 19.4 +/- 13.0 ng/ml in patients with total skin score (TSS) > 20, 8.3 +/- 2.1 ng/ml in patients with TSS < 5, and 6.0 +/- 3.1 ng/ml in volunteers (p = 0.01). NSE platelet lysate concentration was 3.6 +/- 2.9 ng/ml in patients with TSS > 20, 12.4 +/- 4.1 ng/ml in those with TSS < 5, and 14.1 +/- 6.5 ng/ml in healthy individuals (p < 0.001). Volunteers and SSc patients with low TSS had comparable S/PL-NSE index (serum/platelet lysate NSE concentration) (0.42 +/- 0.16 and 0.75 +/- 0.33, respectively), both lower than SSc patients with high TSS (7.45 +/- 5.57) (p < 0.001).
Elevated serum NSE was observed in one-third of SSc patients but not in other autoimmune rheumatic diseases. The inverse relationship between serum and platelet lysate NSE concentration suggests platelet activation as the origin of high serum NSE in SSc. NSE S/PL was the best discriminatory variable between healthy volunteers and SSc patients as well as between patients with high and low TSS. High serum NSE and high NSE-S/PL index seemed to be associated with SSc disease activity. Further work is warranted to investigate a possible role for this marker in assessing disease activity and therapy response.
确定系统性硬化症（SSc）患者血清神经元特异性烯醇化酶（NSE）升高的频率、来源及临床相关性。
采集了75例SSc患者、20例系统性红斑狼疮患者、8例多发性肌炎患者、10例特发性间质性肺病患者及10名健康志愿者的血清。测定了所有个体血清中的NSE水平，以及志愿者和30例SSc患者血小板裂解物中的NSE水平。
26例（34.6%）SSc患者血清NSE升高（平均22.6 ng/ml，范围12.1 - 68.2 ng/ml）。弥漫性SSc患者血清NSE高于局限性疾病患者（分别为16.5±13.4 vs 9.6±5.0 ng/ml，p = 0.006）。未发现血清NSE与肺部或食管受累之间存在关联。病程长的患者血清NSE低于病程短的患者（分别为10.8±7.3 vs 16.1±13.6 ng/ml，p = 0.05）。皮肤总评分（TSS）>20的患者血清NSE为19.4±13.0 ng/ml，TSS<5的患者为8.3±2.1 ng/ml，志愿者为6.0±3.1 ng/ml（p = 0.01）。TSS>20的患者血小板裂解物中NSE浓度为3.6±2.9 ng/ml，TSS<5的患者为12.4±4.1 ng/ml，健康个体为14.1±6.5 ng/ml（p<0.001）。TSS低的志愿者和SSc患者具有相当的S/PL-NSE指数（血清/血小板裂解物NSE浓度）（分别为0.42±0.16和0.75±0.33），均低于TSS高的SSc患者（7.45±5.57）（p<0.001）。
三分之一的SSc患者血清NSE升高，而其他自身免疫性风湿性疾病患者未出现这种情况。血清和血小板裂解物中NSE浓度的反向关系表明血小板活化是SSc患者血清NSE升高的来源。NSE S/PL是区分健康志愿者和SSc患者以及TSS高低患者的最佳变量。高血清NSE和高NSE-S/PL指数似乎与SSc疾病活动相关。有必要进一步研究该标志物在评估疾病活动和治疗反应中的可能作用。
