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一名以咯血为表现的儿科患者的抗中性粒细胞胞浆抗体相关性血管炎

Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in a Pediatric Patient Presenting With Hemoptysis.

作者信息

Anderson Ashley, Bartlett Brian, Chally James, Malicki Joseph

机构信息

Emergency Medicine, Mayo Clinic Health System, Mankato, USA.

出版信息

Cureus. 2024 Sep 4;16(9):e68631. doi: 10.7759/cureus.68631. eCollection 2024 Sep.

Abstract

This case report discusses a rare pediatric case of granulomatosis with polyangiitis (GPA) presenting with hemoptysis, migratory polyarthralgia, significant laboratory abnormalities, and imaging findings. GPA is a form of vasculitis that primarily affects the upper and lower respiratory tracts and the kidneys. Pediatric cases, though rare, offer a distinct set of clinical challenges. The patient presented to the emergency department with hemoptysis, joint pain, and cough. Radiologic findings included diffuse bilateral nodular airspace opacities on chest X-ray (CXR) and ground glass opacities on computed tomography (CT). After hospital admission, the patient's bronchoscopy suggested diffuse alveolar hemorrhage. Laboratory tests were positive for proteinase 3 (PR3), indicating a possible diagnosis of GPA. Further tests, consultations, and evaluations corroborated this diagnosis. Treatment administered included high-dose intravenous steroids, rituximab, and other supportive measures. Pediatric GPA, while rare, is a challenging diagnostic entity. A comprehensive and multidisciplinary approach is pivotal for timely diagnosis and initiation of appropriate therapy.

摘要

本病例报告讨论了一例罕见的小儿肉芽肿性多血管炎(GPA)病例,该病例表现为咯血、游走性多关节痛、显著的实验室异常及影像学表现。GPA是一种血管炎,主要累及上、下呼吸道及肾脏。小儿病例虽罕见,但带来了一系列独特的临床挑战。该患者因咯血、关节疼痛及咳嗽就诊于急诊科。影像学检查结果包括胸部X线(CXR)显示双侧弥漫性结节状气腔混浊,计算机断层扫描(CT)显示磨玻璃影。入院后,患者的支气管镜检查提示弥漫性肺泡出血。实验室检查蛋白酶3(PR3)呈阳性,提示可能诊断为GPA。进一步的检查、会诊及评估证实了这一诊断。所给予的治疗包括大剂量静脉注射类固醇、利妥昔单抗及其他支持措施。小儿GPA虽罕见,但却是一个具有挑战性的诊断实体。全面的多学科方法对于及时诊断及启动恰当治疗至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bc1b/11451306/d41ccb89d968/cureus-0016-00000068631-i01.jpg

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本文引用的文献

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Int J Immunopathol Pharmacol. 2016 Jun;29(2):151-9. doi: 10.1177/0394632015617063. Epub 2015 Dec 18.
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Participation in patient self-management programs.参与患者自我管理项目。
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