肉芽肿病合并多血管炎的儿科表现:双病例研究。

Q3 Medicine
Case Reports in Dermatological Medicine Pub Date : 2025-03-14 eCollection Date: 2025-01-01 DOI:10.1155/crdm/6052518
Marina Handal, Amit Sharma, Mara Ernst, Krystina Khalil, Eduardo Weiss
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引用次数: 0

摘要

肉芽肿病合并多血管炎(GPA)是一种与anca相关的坏死性血管炎,可引起呼吸道和粘膜小血管的肉芽肿性炎症;儿童期GPA罕见,临床上表现为慢性肉芽肿性疾病,或急性血管炎伴快速进行性肺或肾出血。我们报告了两例不同的小儿GPA病例,根据ACR/EULAR GPA标准提供的临床表现和评分指南进行诊断。尽管cANCA结果为阴性,但根据ACR/EULAR GPA诊断标准,患者1的评分为9分。这是基于患者的体格检查,检查显示面部有柔软的结节和斑块,左侧甲壳有结痂性溃疡。左侧前额穿刺活检显示坏死性脉管炎,病理分析显示中性粒细胞为主的炎症浸润和巨细胞。相比之下,患者2的评分为13分,反映了全身性疾病的受累程度,溃疡和结节散布在躯干、四肢和生殖器。实验室检查显示ANCA阳性。此外,该患者在CXR上表现为右侧视神经肉芽肿形成,鼻中隔受损伤的筛窦浸润,双侧腔肿块。由于证据是基于这一独特人口统计学的小队列研究和病例报告,因此缺乏表征儿童GPA的数据。我们报告中的临床表现强调了早期发现疾病和全面检查的必要性,因为及时诊断和最佳治疗方案可能改善小儿GPA患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pediatric Presentations of Granulomatosis With Polyangiitis: A Double Case Study.

Granulomatosis with polyangiitis (GPA) is an ANCA-associated necrotizing vasculitis that causes granulomatous inflammation of small vessels in the respiratory tract and mucosa; GPA in childhood is a rare occurrence that presents distinctly as either a chronic, granulomatous disease that is clinically localized or as an acute vasculitis with rapidly progressive pulmonary or renal hemorrhage. We present two distinct cases of GPA in pediatric patients whose diagnoses were confirmed according to clinical presentation and scoring guidelines offered by the ACR/EULAR GPA Criteria. Despite a negative cANCA result, Patient 1 demonstrated a score of 9 based on the ACR/EULAR criteria for GPA diagnosis. This was based on the patient's physical examination, which revealed tender nodules and plaques along the face as well as a crusted ulceration in the left concha. A punch biopsy of the left lateral forehead revealed necrotizing angiitis with neutrophil-predominant inflammatory infiltrate and giant cells on pathological analysis. In contrast, Patient 2 displayed a score of 13 as reflected in the extent of systemic disease involvement, with ulcerations and nodules scattered along the torso, extremities, and genitalia. Laboratory workup revealed ANCA positivity. Additionally, this patient experienced granuloma formation of the right optic nerve, ethmoid sinus infiltration with damage to the nasal septum, and bilateral cavitary masses on CXR. There is a paucity of data in characterizing GPA in childhood, as evidence is based on small cohort studies and case reports in this unique demographic. The clinical presentations in our report underscore the need for early disease detection and comprehensive workup, as timely diagnosis and optimal treatment regimens may improve the prognoses of pediatric patients with GPA.

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来源期刊
CiteScore
1.50
自引率
0.00%
发文量
24
审稿时长
15 weeks
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