Risk factors for damage in childhood-onset systemic lupus erythematosus - Cumulative disease activity and medication use predict disease damage
Risk factors for damage in childhood-onset systemic lupus erythematosus - Cumulative disease activity and medication use predict disease damage
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DOI：
10.1002/art.10072
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发表时间：
2002-02-01
影响因子：
--
通讯作者：
Feldman, BM
中科院分区：
文献类型：
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作者：
Brunner, HI;Silverman, ED;Feldman, BM
Objective.系统性红斑狼疮国际合作诊所/美国风湿病学会（SLICC/ACR）损伤指数测量成人系统性红斑狼疮（SLE）患者的损伤，但其在儿童期发作的SLE患者中的有用性尚未得到检验。本研究旨在评估SLICC/ACR损伤指数的敏感性，探讨累积疾病活动与儿童期SLE损伤的关系，并确定儿童期SLE损伤的其他危险因素。回顾性评估66例新诊断的儿童期SLE患者的疾病活动和损害，并获得损害的潜在危险因素（年龄、种族、性别、药物、病程、高血压、体重指数、抗磷脂抗体、肾脏疾病、急性血小板减少症）的信息。此外，一组医生进行了调查，以建立SLICC/ACR损伤指数在儿童期发病的SLE的敏感性。SLICC/ACR损伤指数被发现有面子，内容，和结构效度时，用于儿童。患者的平均SLICC/ACR损伤指数评分为1.76（平均随访3.3年）。随着时间的推移累积的疾病活动是损害的单一最佳预测因子（R-2 = 0.30）。其他可能的重要损害危险因素包括皮质类固醇治疗、抗磷脂抗体和急性血小板减少症。免疫抑制剂可能具有保护作用。SLICC/ACR损伤指数，虽然在儿童期发作的SLE有用，可能会受益于引入权重和重新定义的一些项目。持续的疾病活动导致疾病损害，应及时治疗。长期使用高剂量皮质类固醇可能会进一步增加损害，但使用免疫抑制剂可以防止疾病损害;后一项发现可能对儿童期发作的SLE的治疗有潜在意义，值得进一步研究。疾病活动与合并用药之间的关系也需要进一步研究。
Objective. The Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index measures damage in adult patients with systemic lupus erythematosus (SLE), but its usefulness in patients with childhood-onset SLE has not been examined. This study was conducted to evaluate the sensibility of the SLICC/ACR Damage Index, to investigate how cumulative disease activity is related to damage in childhood-onset SLE, and to identify other risk factors for damage in childhood-onset SLE.Methods. Disease activity and damage in 66 patients with newly diagnosed childhood-onset SLE were assessed retrospectively, and information on potential risk factors for damage (age, race, sex, medications, duration of disease, hypertension, body mass index, anti phospholipid antibodies, kidney disease, acute thrombocytopenia) was obtained. In addition, a group of physicians was surveyed to establish the sensibility of the SLICC/ACR Damage Index in childhood-onset SLE.Results. The SLICC/ACR Damage Index was found to have face, content, and construct validity when used in children. The mean SLICC/ACR Damage Index score of the patients was 1.76 (mean follow-up 3.3 years). Cumulative disease activity over time was the single best predictor of damage (R-2 = 0.30). Other, possibly, important risk factors for damage were corticosteroid treatment, the presence of antiphospholipid antibodies, and acute thrombocytopenia. It was determined that immunosuppressive agents may be protective.Conclusion. The SLICC/ACR Damage Index, though useful in childhood-onset SLE, may benefit from the introduction of weightings and redefinition of some of the items. Ongoing disease activity leads to disease damage, and treatment should be prompt. Prolonged use of high-dose corticosteroids may further increase damage, but use of immunosuppressive agents may protect against disease damage; this latter finding may have potential implications for the treatment of childhood-onset SLE and deserves further study. The relationship between disease activity and concomitant use of medication also requires further investigation.