Anderson Ashley, Bartlett Brian, Chally James, Malicki Joseph
Emergency Medicine, Mayo Clinic Health System, Mankato, USA.
Cureus. 2024 Sep 4;16(9):e68631. doi: 10.7759/cureus.68631. eCollection 2024 Sep.
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.
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。