Jari Mohsen
Department of Pediatric Rheumatology, Imam Hossein Children's Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.
Case Rep Hematol. 2021 Mar 3;2021:5520258. doi: 10.1155/2021/5520258. eCollection 2021.
Granulomatosis with polyangiitis disease (GPA) is a rare vasculitis characterized by granulomatous inflammation of respiratory tracts, glomerulonephritis, and vasculitis of other organs. . A 13-year-old girl was referred due to swelling and pain on her left arm. The Doppler and compression ultrasonography showed noncompressible left brachial and axillary vein thrombosis. Sinus computed tomography (CT) demonstrated pansinusitis, and spiral chest CT showed alveolar hemorrhage. Laboratory tests showed hematuria, proteinuria, and highly positive antineutrophil cytoplasmic antibody (cANCA). Laboratory tests of coagulopathy were normal. The patient was recognized as a case of GPA.
Although GPA is not frequently associated with thrombosis especially in children, this is the first report that shows thrombosis may be the first manifestation of GPA in an adolescent.
肉芽肿性多血管炎（GPA）是一种罕见的血管炎，其特征为呼吸道的肉芽肿性炎症、肾小球肾炎以及其他器官的血管炎。一名13岁女孩因左臂肿胀疼痛前来就诊。多普勒及压迫超声检查显示左侧肱静脉和腋静脉血栓形成，无法被压迫。鼻窦计算机断层扫描（CT）显示全鼻窦炎，胸部螺旋CT显示肺泡出血。实验室检查显示血尿、蛋白尿，抗中性粒细胞胞浆抗体（cANCA）高度阳性。凝血功能障碍的实验室检查结果正常。该患者被诊断为GPA病例。
尽管GPA尤其是在儿童中并不常伴有血栓形成，但这是首例显示血栓形成可能是青少年GPA首发表现的报告。
