González B, Hernández P, Olguín H, Miranda M, Lira L, Toso M, Quezada A, Norambuena X, Talesnik E, Méndez C, Navarrete C
Pediatric Rheumatology and Immunology Unit, Luis Calvo Mackenna Hospital, Santiago, Chile.
Lupus. 2005;14(11):918-23. doi: 10.1191/0961203303lu2183xx.
The objective of this study was to analyse the survival rate and cause of death in children with systemic lupus erythematosus (SLE) during the past 30 years in Chile. A retrospective analysis was performed between 1969 and 2000 on patients attending pediatric rheumatology centres in Santiago, Chile. Survival and causes of death in 31 children followed from 1969 to 1980 fulfilling the 1982 American College of Rheumatology criteria for SLE and treated with oral steroids were compared with 50 other patients who were treated with oral steroids and an aggressive treatment of IV bolus of cyclophosphamide (38 patients) and azathioprine (12 patients). Global survival at five and 10 years follow-up for the patients studied from 1969 to 1980 was 68 and 40%, respectively. During the second study period these values were significantly improved and global survival reached 95% at five years and 90% at 10 years follow-up (P < 0.05). Survival at 10 years follow-up for patients with lupus nephropathy increased from 28% (study period 1964-1980) to 86% (study period 1984-2000). Twelve children died (38%) during the 1964-1980 study period. The causes of death were six due to kidney failure, three due to infectious conditions and another three of unknown causes. During the 1980-2000 study period mortality reached 6% (three cases), two cases died of a lupus flare-up and one case due to infection. In the last three decades, we have seen an important increase in the survival of children with SLE, especially in those patients with renal involvement. Management with immunosuppressive drugs, such as IV cyclophosphamide or azathioprine has changed the prognosis in these children. These results demonstrate that our children with SLE increased their life expectancy but are now faced with new types of morbidity because of the sequelae related to the disease itself.
本研究的目的是分析过去30年里智利系统性红斑狼疮（SLE）患儿的生存率及死亡原因。对1969年至2000年间在智利圣地亚哥儿科风湿病中心就诊的患者进行了回顾性分析。将1969年至1980年期间符合1982年美国风湿病学会SLE标准并接受口服类固醇治疗的31名患儿的生存情况及死亡原因，与另外50名接受口服类固醇以及环磷酰胺静脉推注（38例）和硫唑嘌呤（12例）积极治疗的患者进行了比较。1969年至1980年期间所研究患者在5年和10年随访时的总体生存率分别为68%和40%。在第二个研究期间，这些数值显著改善，5年随访时总体生存率达到95%，10年随访时为90%（P<0.05）。狼疮性肾病患者在10年随访时的生存率从28%（1964 - 1980年研究期）提高到86%（1984 - 2000年研究期）。在1964 - 1980年研究期间，12名患儿死亡（38%）。死亡原因是6例肾衰竭、3例感染性疾病以及另外3例原因不明。在1980 - 2000年研究期间，死亡率为6%（3例），2例死于狼疮病情突然加重，1例死于感染。在过去三十年中，我们看到SLE患儿的生存率有了显著提高，尤其是那些有肾脏受累的患者。使用免疫抑制药物如静脉注射环磷酰胺或硫唑嘌呤进行治疗改变了这些患儿的预后。这些结果表明，我们的SLE患儿预期寿命增加了，但由于与疾病本身相关的后遗症，现在面临着新型的发病情况。
