Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus
Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus
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DOI：
10.1002/art.23204
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发表时间：
2008-02-01
影响因子：
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通讯作者：
Silverman, Earl D.
中科院分区：
文献类型：
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作者：
Brunner, Hermine I.;Gladman, Dafna D.;Silverman, Earl D.
目的：研究儿童期发病的系统性红斑狼疮（SLE）与成人期发病的系统性红斑狼疮之间可能存在的差异。
方法：将一个儿童期发病的SLE起始队列（n = 67）与一个成人期发病的SLE起始队列（n = 131）进行比较，这些患者均在1990年至1998年期间确诊，并随访至1999年2月。前瞻性信息包括药物使用数据、实验室指标以及分别由SLE疾病活动指数（SLEDAI）和系统性红斑狼疮国际临床协作组/美国风湿病学会损伤指数（SDI）所衡量的疾病活动度和损伤情况。
结果：85%的儿童期发病的SLE患者和88%的成人期发病的SLE患者为女性；平均随访时间分别为3.2年和3.5年。平均而言，在确诊时以及随访期间，儿童的疾病活动度比成人更高。儿童期发病的SLE患者肾脏疾病的发病率更高（儿童为78%，成人则为52%；P = 0.0005），并且调整后的儿童SLEDAI肾脏评分均值高于成人（2.37比0.82；P < 0.0001）。SLE儿童使用类固醇（97%比72%；P < 0.0001）和免疫抑制药物（66%比37%；P = 0.0001）治疗的频率明显更高。在随访期间有4名成人SLE患者死亡，但儿童无死亡病例。在随访结束时，儿童期发病的SLE患者的平均SDI评分高于成人期发病的SLE患者（1.70比0.76；P = 0.008）。
结论：儿童期发病的SLE儿童在发病时以及随着时间推移，其疾病活动度比成人SLE患者更高，尤其是肾脏疾病活动度。与成人SLE患者相比，儿童接受更强化的药物治疗，并且遭受更多损伤，这些损伤通常与类固醇毒性有关。
Objective. To investigate potential differences between childhood-onset and adult-onset systemic lupus erythematosus (SLE).Methods. An inception cohort with childhood-onset SLE (n = 67) was compared with an inception cohort with adult-onset SLE (n = 131), each of whom was diagnosed between 1990 and 1998 and followed up until February 1999. Prospective information included data on medications, laboratory markers, and disease activity and damage as measured by the SLE Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), respectively.Results. Eighty-five percent of patients with childhood-onset SLE and 88% of patients with adult-onset SLE were female; the mean duration of followup was 3.2 and 3.5 years, respectively. On average, the children had more-active disease than did the adults at the time of diagnosis and during followup. There was a higher incidence of renal disease in those with childhood-onset SLE (78% versus 52% in adults; P = 0.0005), and the adjusted mean SLEDAI renal score was higher in the children than in the adults (2.37 versus 0.82; P < 0.0001). Treatment with steroids (97% versus 72%; P < 0.0001) and immunosuppressive drugs (66% versus 37%; P = 0.0001) was used significantly more often in children with SLE. Four adult patients with SLE, but none of the children, died during the followup. At the end of the followup, the mean SDI scores in those with childhood-onset SLE were higher than those with adult-onset SLE (1.70 versus 0.76; P = 0.008).Conclusion. Children with childhood-onset SLE have more active disease at presentation and over time than do adults with SLE, especially active renal disease. Compared with adults with SLE, children receive more intensive drug therapy and accrue more damage, often related to steroid toxicity.