Renal and cardiopulmonary manifestations in patients with juvenile systemic lupus erythematosus: Clinical experience in Cali, Colombia

María Paula Rengifo Mosquera, Robinson Pacheco, María del Pilar Gómez Mora
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Abstract

Introduction

Juvenile systemic lupus erythematosus (jSLE) is a chronic, multisystemic autoimmune disease with a variable course and prognosis. The pediatric population accounts for 20% of total SLE cases. Renal and cardiopulmonary manifestations contribute significantly to morbidity and mortality.

Objective

To clinically and demographically characterize the renal and cardiopulmonary manifestations of jSLE in the study population.

Materials and methods

A cross-sectional observational study was conducted in patients with jSLE (EULAR/ACR-2019 criteria) at a Pediatric Rheumatology clinic in Cali, Colombia, between 2012 and 2024. Data were collected from medical records and analyzed using the IBM SPSS v29 statistical program.

Results

Among 98 patients with JSLE, 30.6% had renal involvement, 10.2% had cardiopulmonary involvement, and 6.1% had both. Regarding renal involvement, 83.3% were female, with a mean age of 11.4 years; 76.6% presented at disease onset, while 13.3% developed renal involvement more than two years after diagnosis. Most patients presented with proteinuria, hypertension, and nephrotic syndrome. Renal biopsy was performed in 73.3% of cases, revealing nephritis types 2, 3, and 4 (ISN/RPS classification) in 13.3%, 10%, and 50% of patients, respectively. The mean activity index was 9/24, and the chronicity index was 2/12. Regarding cardiopulmonary involvement, 90% of patients were female, with a mean age of 12.5 years; 90% presented at diagnosis. The most common clinical manifestations were chest pain, pleuritis, and pericarditis. Laboratory findings in renal involvement included anemia, elevated erythrocyte sedimentation rate (ESR), decreased complement levels (C3 and C4), and positive Anti-DNA and Anti-SM antibodies. In cardiopulmonary involvement, findings included elevated C-reactive protein and ESR, decreased C3 and C4 levels, and positive Anti-DNA, Anti-SM, and Anti-RNP antibodies. Additionally, 40% of patients with renal involvement and 60% of those with cardiopulmonary involvement had other autoimmune diseases. Mortality occurred in one patient with mixed involvement.

Conclusion

In jSLE, renal involvement is a common presentation at disease onset, while cardiopulmonary involvement is less prevalent. Despite the severity of these manifestations, mortality was low. However, the high inflammatory activity and significant percentage of autoimmune comorbidities highlight the complexity of this condition and underscore the importance of early identification and treatment to improve the quality of life of this population.
少年系统性红斑狼疮患者的肾脏和心肺表现:哥伦比亚卡利的临床经验
青少年系统性红斑狼疮(jSLE)是一种病程和预后多变的慢性多系统自身免疫性疾病。小儿占SLE总病例的20%。肾脏和心肺表现是发病率和死亡率的重要因素。目的对研究人群中jSLE的肾脏和心肺表现进行临床和人口学特征分析。材料和方法2012年至2024年,在哥伦比亚卡利的一家儿科风湿病诊所对jSLE (EULAR/ACR-2019标准)患者进行了一项横断面观察性研究。从医疗记录中收集数据,并使用IBM SPSS v29统计程序进行分析。结果98例JSLE患者中,30.6%有肾脏受累,10.2%有心肺受累,6.1%两者兼有。在肾脏受累方面,83.3%为女性,平均年龄11.4岁;76.6%的患者在发病时出现,13.3%的患者在诊断后两年多出现肾脏受累。大多数患者表现为蛋白尿、高血压和肾病综合征。73.3%的病例行肾活检,分别有13.3%、10%和50%的患者显示2型、3型和4型肾炎(ISN/RPS分类)。平均活动性指数为9/24,慢性性指数为2/12。在心肺受累方面,90%的患者为女性,平均年龄12.5岁;90%在诊断时出现。最常见的临床表现为胸痛、胸膜炎、心包炎。肾脏受累的实验室结果包括贫血,红细胞沉降率(ESR)升高,补体水平(C3和C4)降低,抗dna和抗sm抗体阳性。在心肺受累方面,发现包括c反应蛋白和ESR升高,C3和C4水平降低,抗dna,抗sm和抗rnp抗体阳性。此外,40%的肾脏受累患者和60%的心肺受累患者有其他自身免疫性疾病。1例混合性发病患者死亡。结论在jSLE中,肾脏受累是发病时常见的表现,而心肺受累则不常见。尽管这些表现很严重,但死亡率很低。然而,高炎症活性和显著比例的自身免疫性合并症突出了这种疾病的复杂性,并强调了早期识别和治疗对提高这一人群生活质量的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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