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小儿显微镜下多血管炎伴持续性咳嗽和咯血:1例报告并文献复习

Microscopic polyangiitis presenting with persistent cough and hemoptysis in pediatrics: A case report and review of the literature.

作者信息

Zhu Yantong, Zheng Xiangrong

机构信息

Department of Pediatrics, Xiangya Hospital, Central South University, Changsha, China.

出版信息

Front Oncol. 2022 Dec 6;12:987507. doi: 10.3389/fonc.2022.987507. eCollection 2022.

Abstract

BACKGROUND

Microscopic polyangiitis (MPA) is a necrotizing vasculitis that involves small- and medium-sized vessels and is associated with the presence of antineutrophil cytoplasmic antibodies with a perinuclear staining pattern (p-ANCA). The kidney and lungs are the organs primarily affected. MPA is rare in children and is easily misdiagnosed. Below is a complete case history of the course of the disease.

CASE PRESENTATION

An 11-year-old girl with a 1-month history of cough and hemoptysis showed no improvement after imipenem-cilastatin treatment. p-ANCA and microscopic hematuria and proteinuria were positive, and a chest CT revealed an area of shadow in the bilateral lower lobe of the lungs. Renal biopsies showed crescentic glomerulonephritis, and MPA was diagnosed based on these criteria. The patient exhibited dramatic clinical and imaging improvements after immunosuppressive treatment.

CONCLUSION

The organs most commonly involved in MPA in children are the lungs, kidneys, skin, nervous system organs, and organs of the gastrointestinal tract. Careful examination should be carried out in these patients while biopsies of the kidney or any other organs remain the gold standard for diagnostic purposes. Pulmonary involvement may be the initial symptom of the disease and should not be confused with pneumonia. A urinalysis should be performed in patients with hemoptysis. Antibiotics should be used with caution.

摘要

背景

显微镜下多血管炎(MPA)是一种累及中小血管的坏死性血管炎,与核周型抗中性粒细胞胞浆抗体(p-ANCA)阳性有关。肾脏和肺是主要受累器官。MPA在儿童中罕见,且容易误诊。以下是该疾病病程的完整病例报告。

病例介绍

一名11岁女孩,有1个月咳嗽和咯血病史,接受亚胺培南-西司他丁治疗后无改善。p-ANCA、镜下血尿和蛋白尿均呈阳性,胸部CT显示双肺下叶有阴影区。肾活检显示新月体性肾小球肾炎,根据这些标准诊断为MPA。患者在接受免疫抑制治疗后临床和影像学表现有显著改善。

结论

儿童MPA最常累及的器官是肺、肾、皮肤、神经系统器官和胃肠道器官。对这些患者应进行仔细检查,而肾活检或任何其他器官的活检仍是诊断的金标准。肺部受累可能是该病的首发症状,不应与肺炎混淆。咯血患者应进行尿液分析。抗生素应谨慎使用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a770/9763891/290b2c5a8b9b/fonc-12-987507-g001.jpg

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