Bloom B J, Tucker L B, Miller L C, Schaller J G
Division of Ambulatory Pediatrics, Hasbro Children's Hospital at Rhode Island Hospital and Brown University School of Medicine, Providence 02903, USA.
J Rheumatol. 1998 Aug;25(8):1620-5.
To study the prevalence of coagulation abnormalities in children with systemic juvenile rheumatoid arthritis (JRA) using a sensitive marker of fibrin degradation, and to determine whether serial levels of this variable parallel disease activity or predict response to medications in this disease.
Levels of d-dimer were determined in 24 consecutive patients with systemic JRA in conjunction with complete blood counts, erythrocyte sedimentation rate, maximum fever, duration of morning stiffness, and swollen joint count. Serial levels were then obtained in 11 patients. Linear regression analyses were done to determine any correlations between d-dimer and the other variables; and paired t test was used to compare levels before and after treatment interventions. Levels of d-dimer were also compared against concurrent clinical events such as pericarditis.
Elevated levels of d-dimer were found in 23/24 of the patients (96%). When serial levels were analyzed, there were correlations between levels of d-dimer and fever (p = 0.03) and total leukocyte count (p = 0.04), but not with other variables. There was a significant reduction in levels before and after treatment in patients deemed to be clinical responders to immunomodulatory agents (p = 0.02). Elevated levels were also indicative of severe disease over the remainder of followup; lack of d-dimer indicated a benign disease course.
With the use of a sensitive and specific marker of fibrinolysis known as d-dimer, coagulation abnormalities were more prevalent in children with systemic JRA than previously reported, and are frequently found during periods of active disease. Furthermore, serial levels of d-dimer appear to parallel response to disease modifying agents, and may predict outcome over a short followup period. Fibrin d-dimer may represent a novel marker that, when used in combination with known variables, could enhance that assessment of disease activity and response to medications in children with systemic onset JRA.
使用纤维蛋白降解的敏感标志物研究全身型幼年类风湿关节炎（JRA）患儿凝血异常的患病率，并确定该变量的系列水平是否与疾病活动度平行或预测该疾病对药物的反应。
对24例连续的全身型JRA患者测定D-二聚体水平，并同时检测全血细胞计数、红细胞沉降率、最高体温、晨僵持续时间和关节肿胀计数。然后对11例患者进行系列水平检测。进行线性回归分析以确定D-二聚体与其他变量之间的任何相关性；并使用配对t检验比较治疗干预前后的水平。还将D-二聚体水平与并发的临床事件如心包炎进行比较。
24例患者中有23例（96%）D-二聚体水平升高。分析系列水平时，D-二聚体水平与发热（p = 0.03）和白细胞总数（p = 0.04）之间存在相关性，但与其他变量无关。被认为对免疫调节药物有临床反应的患者治疗前后水平有显著降低（p = 0.02）。升高的水平也表明在随访的其余时间疾病严重；D-二聚体水平正常表明疾病进程良性。
使用一种称为D-二聚体的敏感且特异的纤维蛋白溶解标志物，全身型JRA患儿的凝血异常比以前报道的更普遍，并且在疾病活动期经常出现。此外，D-二聚体的系列水平似乎与疾病改善药物的反应平行，并且可能在短期随访期预测结果。纤维蛋白D-二聚体可能代表一种新的标志物，当与已知变量结合使用时，可以增强对全身型JRA患儿疾病活动度和药物反应的评估。
