儿童系统性红斑狼疮（SLE）：34例患者的临床和免疫学发现分析及与成人SLE特征的比较
Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults.
作者信息
Font J, Cervera R, Espinosa G, Pallarés L, Ramos-Casals M, Jiménez S, García-Carrasco M, Seisdedos L, Ingelmo M
机构信息
Department of Medicine, IDIBAPS, Hospital Clinic, School of Medicine, University of Barcelona, Catalonia, Spain.
出版信息
Ann Rheum Dis. 1998 Aug;57(8):456-9. doi: 10.1136/ard.57.8.456.
OBJECTIVE
To define the pattern of disease expression in patients with childhood onset systemic lupus erythematosus (SLE).
METHODS
Prospective analysis of clinical manifestations and immunological features of 34 patients in whom the first manifestations appeared in childhood from a series of 430 unselected patients with SLE.
RESULTS
Thirty one (91%) patients from the childhood onset group were female and three male (9%) (ratio female/male, 10/1, with no difference compared with the adult onset group). Mean age of this group at disease onset was 11 years (range 5-14) compared with 32 years (15-48) for the remaining patients. The childhood onset patients more often had nephropathy (20% v 9% in adult onset SLE, p = 0.04; OR:2.7; 95% CI:1.1, 7), fever (41% v 21%, p = 0.006; OR:2.6, 95% CI:1.2, 5.7), and lymphadenopathy (6% v 0.5%, p = 0.03, OR: 12.3, 95% CI: 1.2, 127.6), as presenting clinical manifestations. During the evolution of the disease, the childhood onset patients had an increased prevalence of malar rash (79% v 51%, p = 0.002; OR:3.7; 95% CI:1.5, 9.5) and chorea (9% v 0%, p < 0.0001). This group exhibited a higher prevalence of anticardiolipin antibodies (aCL) of the IgG isotype when compared with the remaining patients (29% v 13%, p = 0.017; OR:2.9, 95% CI:1.2, 6.8). No significant differences were found among the other antibodies between the two groups. Childhood onset patients more often received azathioprine (15% v 6%, p = 0.00004; OR:11.2; 95% CI:2.8, 44.9) but no differences were detected between the groups concerning side effects or drug toxicity.
CONCLUSIONS
The presentation and the clinical course of SLE varied in this series of 430 patients depending on their age at disease onset. Nephropathy, fever, and lymphadenopathy were more common in childhood onset patients as presenting clinical manifestations, while malar rash, chorea, and detection of IgG aCL were more common during the evolution of the disease.
目的
明确儿童期起病的系统性红斑狼疮（SLE）患者的疾病表现模式。
方法
对430例未经挑选的SLE患者中首次发病于儿童期的34例患者的临床表现和免疫学特征进行前瞻性分析。
结果
儿童期起病组31例（91%）为女性，3例（9%）为男性（女性/男性比例为10/1，与成人起病组无差异）。该组患者发病时的平均年龄为11岁（范围5 - 14岁），其余患者为32岁（15 - 48岁）。儿童期起病的患者更常出现肾病（成人起病的SLE中为20% 对9%，p = 0.04；OR：2.7；95% CI：1.1，7）、发热（41% 对21%，p = 0.006；OR：2.6，95% CI：1.2，5.7）和淋巴结病（6% 对0.5%，p = 0.03，OR：12.3，95% CI：1.2，127.6）作为首发临床表现。在疾病进展过程中，儿童期起病的患者出现颊部皮疹（79% 对51%，p = 0.002；OR：3.7；95% CI：1.5，9.5）和舞蹈病（9% 对0%，p < 0.0001）的患病率增加。与其余患者相比，该组IgG同种型抗心磷脂抗体（aCL）的患病率更高（29% 对13%，p = 0.017；OR：2.9，95% CI：1.2，6.8）。两组之间其他抗体未发现显著差异。儿童期起病的患者更常接受硫唑嘌呤治疗（15% 对6%，p = 0.00004；OR：11.2；95% CI：2.8，44.9），但两组在副作用或药物毒性方面未检测到差异。
结论
在这430例患者系列中，SLE的表现和临床病程因发病年龄而异。肾病、发热和淋巴结病在儿童期起病的患者中作为首发临床表现更为常见，而颊部皮疹、舞蹈病和IgG aCL的检测在疾病进展过程中更为常见。
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