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A case of infantile systemic lupus erythematosus with severe lupus nephritis and EBV infection

机译：婴幼儿系统性红斑狼疮合并严重狼疮肾炎和EBV感染1例

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摘要

Infantile systemic lupus erythematosus (iSLE) is extremely rare. Patients with iSLE usually become severely unwell and have poor prognosis. Epstein–Barr virus (EBV) infection has been implicated in the development of SLE in both adults and children. Recently, we experienced a case of iSLE with severe lupus nephritis (LN) and EBV infection. A 14-month-old Japanese boy was diagnosed with iSLE according to the American Rheumatism Association criteria. Renal biopsy showed LN classified as International Society of Nephrology/Renal Pathology Society class IV-G (A), and liver biopsy showed lupus hepatitis. Steroid pulse treatment resulted in improvement of the levels of serological markers of SLE such as double-stranded DNA and complement, but his proteinuria worsened and he developed acute nephritic–nephrotic syndrome. Monthly intravenous cyclophosphamide (IVCY) therapy dramatically reduced his proteinuria and led to complete remission (urinary protein/creatinine ratio <0.1 mg/mg), with gradual improvement in levels of serological markers. EBV antibody titers and EBV polymerase chain reaction (PCR) of peripheral blood lymphocytes suggested that the onset of iSLE might have been associated with EBV infection. At his 2-year follow-up visit, he was healthy and remained in complete remission. We conclude that IVCY treatment might be well tolerated and effective in cases of iSLE. EBV infection might play an important role in the pathogenesis of iSLE.
机译：婴儿系统性红斑狼疮（iSLE）极为罕见。 iSLE患者通常会变得严重不适，预后也较差。在成人和儿童中，爱泼斯坦-巴尔病毒（EBV）感染都与SLE的发展有关。最近，我们经历了一例iSLE并伴有严重狼疮性肾炎（LN）和EBV感染。根据美国风湿病协会的标准，一名14个月大的日本男孩被诊断​​出患有iSLE。肾活检显示LN被归类为国际肾脏病学会/肾脏病理学会IV-G级（A），肝活检显示狼疮性肝炎。类固醇脉冲治疗可改善SLE的血清学指标，例如双链DNA和补体，但他的蛋白尿恶化，并发展为急性肾病-肾病综合征。每月静脉给予环磷酰胺（IVCY）治疗可显着降低他的蛋白尿并导致完全缓解（尿蛋白/肌酐比值<0.1 mg / mg），血清学指标水平逐渐提高。 EBV抗体滴度和外周血淋巴细胞的EBV聚合酶链反应（PCR）表明，iSLE的发作可能与EBV感染有关。在为期2年的随访中，他身体健康，完全缓解。我们得出的结论是，对于iSLE，IVCY治疗可能耐受良好且有效。 EBV感染可能在iSLE的发病机理中起重要作用。

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  • 来源
    《CEN Case Reports》 |2013年第2期|190-193|共4页
  • 作者

    Natsuko Kishi; Kenichi Suga; Sato Matsuura; Yukiko Kinoshita; Maki Urushihara; Shuji Kondo; Etsuko Kitano; Michiyo Hatanaka; Hajime Kitamura; Tetsuya Sato; Akihiko Maeda; Shoji Kagami;

  • 作者单位

    Department of Pediatrics Institution of Health Biosciences The University of Tokushima Graduate School">(1);

    Department of Pediatrics Institution of Health Biosciences The University of Tokushima Graduate School">(1);

    Department of Pediatrics Tokushima Prefectural Central Hospital">(6);

    Department of Pediatrics Institution of Health Biosciences The University of Tokushima Graduate School">(1);

    Department of Pediatrics Institution of Health Biosciences The University of Tokushima Graduate School">(1);

    Department of Pediatrics Institution of Health Biosciences The University of Tokushima Graduate School">(1);

    Department of Pediatrics Institution of Health Biosciences The University of Tokushima Graduate School">(1);

    Department of Medical Technology Kobe Tokiwa University">(2);

    Department of Medical Technology Kobe Tokiwa University">(2);

    Department of Nutritional Sciences for Well-being Kansai University of Welfare Sciences">(3);

    Department of Pediatrics Kochi Medical School">(4);

    Department of Pediatrics Kochi Prefectural Hatakenmin Hospital">(5);

    Department of Pediatrics Institution of Health Biosciences The University of Tokushima Graduate School">(1);

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  • 关键词

    Infant; Intravenous cyclophosphamide; Lupus nephritis; Systemic lupus erythematosus; Epstein–Barr virus;

    机译：婴儿;静脉内环磷酰胺狼疮性肾炎;系统性红斑狼疮;爱泼斯坦巴尔病毒;

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