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儿童期起病的系统性红斑狼疮:患者特征及其向成年期的转变。

Childhood-onset Systemic Lupus Erythematosus: Patients features and their transition into adulthood.

作者信息

Valenzuela Paula, Ladino Mabel, Vargas Nelson

机构信息

Hospital Félix Bulnes, Santiago, Chile.

Hospital San Juan de Dios, Santiago, Chile.

出版信息

Andes Pediatr. 2021 Jun;92(3):375-381. doi: 10.32641/andespediatr.v92i3.1653.

DOI:10.32641/andespediatr.v92i3.1653
PMID:34479243
Abstract

INTRODUCTION

Systemic Lupus Erythematosus (SLE) is an autoimmune, multisystemic, chronic disease that is diffi cult to diagnose. Few studies describe its features in the South American pediatric population. Ob jective: to describe clinical and laboratory features, course, and treatment of childhood-onset SLE patients and their transition into adulthood.

PATIENTS AND METHOD

Retrospective study of patients diagnosed with SLE in a Children's Rheumatology Unit of a hospital in Santiago de Chile between 2001 and 2017. Epidemiological, clinical, laboratory, treatment received, evolution, complications and hospitalizations data were registered. It was considered severe SLE the cases with renal or cen tral nervous system involvement.

RESULTS

31 patients were studied, all with the disease longer than 6 months. The female/male ratio was 5.2/1. The median age of presentation was 12.5 years. In 94% of cases, the diagnostic delay was less than 6 months. The most frequent clinical characteristics were arthritis (87%), skin lesions (58%), and renal involvement (58%). The most frequent laboratory findings were positive antinuclear antibodies (100%), positive anti-dsDNA antibodies (74%), and hypocomplementemia (71%). Corticosteroids, hydroxychloroquine, and mycophenolate were the most commonly used drugs. There was no mortality in this group. 97% of patients had "satisfactory check-ups" during pediatric care and 59% in the adult one. The transition was scheduled in most cases.

CONCLUSIONS

The results of this study were similar to other publications and is one of the few studies describing SLE in the Chilean pediatric population. In addition, it describes the transition into adulthood.

摘要

引言

系统性红斑狼疮(SLE)是一种自身免疫性、多系统慢性疾病,诊断困难。很少有研究描述其在南美儿科人群中的特征。目的:描述儿童期起病的SLE患者的临床和实验室特征、病程及治疗情况,以及他们向成年期的过渡。

患者与方法

对2001年至2017年期间在智利圣地亚哥一家医院的儿童风湿病科诊断为SLE的患者进行回顾性研究。记录流行病学、临床、实验室、接受的治疗、病情演变、并发症及住院数据。将累及肾脏或中枢神经系统的病例视为重症SLE。

结果

共研究了31例患者,所有患者病程均超过6个月。男女比例为5.2∶1。发病的中位年龄为12.5岁。94%的病例诊断延迟少于6个月。最常见的临床特征为关节炎(87%)、皮肤病变(58%)和肾脏受累(58%)。最常见的实验室检查结果为抗核抗体阳性(100%)、抗双链DNA抗体阳性(74%)和补体降低(71%)。糖皮质激素、羟氯喹和霉酚酸酯是最常用的药物。该组无死亡病例。97%的患者在儿科治疗期间“检查结果满意”,在成人治疗期间这一比例为59%。大多数病例的过渡是按计划进行的。

结论

本研究结果与其他文献相似,是少数描述智利儿科人群中SLE的研究之一。此外,它还描述了向成年期的过渡。

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