Influence of age at disease onset in the outcome of paediatric systemic lupus erythematosus
Influence of age at disease onset in the outcome of paediatric systemic lupus erythematosus
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DOI：
10.1093/rheumatology/kep067
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发表时间：
2009-07-01
期刊：
影响因子：
5.5
通讯作者：
Cimaz, Rolando
中科院分区：
文献类型：
--
作者：
Descloux, Elodie;Durieu, Isabelle;Cimaz, Rolando
方法。对 56 名 pSLE 患者进行了研究，分为三组（青春期前、青春期前后和青春期后发病）。使用Fishers精确检验和KruskalWallis检验比较SDI（SLE的SLICC/ACR损伤指数）、患者特征、疾病表现和治疗。构建 KaplanMeier 曲线来比较损坏发生的风险。结果。当发病年龄增加时，损伤风险 (SDI 1) 显着降低（青春期前 pSLE 为 89，青春期周围 pSLE 为 57，青春期后 pSLE 为 38）。在研究的所有疾病持续时间间隔（13、35、58、810、10 年）和随访结束时都发现了这种过度风险。 KaplanMeier 曲线表明年轻患者受损的风险更高且更早。年幼的儿童表现出更高频率的自身免疫家族史。神经精神疾病和损害的发生率随着发病年龄的增加而降低（P<0.05）。当发病年龄降低时，大剂量泼尼松（0.5 mg/kg/天）的累积持续时间和似乎导致损害的免疫抑制药物的使用数量显着增加。结论。 pSLE 损伤的风险与发病年龄呈负相关。年幼儿童中观察到的较差结果可能是由于更严重的疾病表现、更高的感染易感性和更积极的治疗，特别是在病程的前 6 个月内。
Methods. Fifty-six patients with pSLE, divided into three groups (pre-pubertal, peripubertal and post-pubertal onset), were studied. The SDI (SLICC/ACR Damage Index for SLE), patients characteristics, disease manifestations and treatments were compared using Fishers exact test and KruskalWallis test. KaplanMeier curves were constructed to compare the risk of damage occurrence.Results. The risk of damage (SDI 1) significantly decreased when age at disease onset increased (89 in pre-pubertal pSLE, 57 in peripubertal pSLE and 38 in post-pubertal pSLE). This excess of risk was found in all disease duration intervals studied (13, 35, 58, 810, 10 years) and at the end of follow-up. KaplanMeier curves indicated a higher and earlier risk of damage in younger patients. Young children showed higher frequency of autoimmune family history. The frequency of neuropsychiatric disorders and damages decreased with age at disease onset (P 0.05). Cumulative duration of high-dose prednisone ( 0.5 mg/kg/day) and number of immunosuppressive drugs used that seem to contribute to damage significantly increased when age at disease onset decreased.Conclusions. The risk of damage is inversely correlated with age at disease onset in pSLE. The poorer outcome observed in younger children may be explained by a more severe disease expression, may be a higher infectious susceptibility, and a more aggressive therapy, particularly within the first 6 months of disease course.