本文报道1例6岁高IgE综合征（hyperimmunoglobulin E syndrome，HIES）合并肺动脉假性动脉瘤（pulmonary artery pseudoaneurysm，PAPA）患儿的诊治经过。患儿因咯血入院，胸部CT血管成像（computed tomography angiography，CTA）明确诊断为PAPA。介入术中虽行体动脉及肺动脉主干造影均未显示瘤体，但通过微导管超选择性左下肺动脉分支造影成功显示瘤腔，并予弹簧圈栓塞，术后咯血停止。患儿于术后3个月接受非亲缘造血干细胞移植，随访18个月无咯血复发，影像学检查示弹簧圈位置稳定、PAPA无复发。本病例提示，HIES合并咯血的儿童应高度警惕PAPA可能，介入治疗需联合体动脉及多级肺动脉造影以精准定位，积极治疗原发病是预防复发的关键。

This case report describes the diagnosis and treatment of a 6-year-old child with hyperimmunoglobulin E syndrome (HIES) complicated by pulmonary artery pseudoaneurysm (PAPA). The child presented with hemoptysis, and chest computed tomography angiography (CTA) confirmed the diagnosis of PAPA. During interventional embolization, neither systemic arteriography nor main pulmonary artery angiography revealed the aneurysm; however, superselective angiography of the left lower pulmonary artery branch via a microcatheter successfully visualized the aneurysmal sac, which was then embolized with coils. Hemoptysis ceased after the procedure. The child underwent unrelated hematopoietic stem cell transplantation three months later and has remained free of hemoptysis during 18 months of follow-up, with imaging showing stable coil position and no recurrence of PAPA. This case highlights that in children with HIES presenting with hemoptysis, PAPA should be highly suspected. Interventional treatment requires combined systemic and multi-level pulmonary artery angiography for accurate localization, and active management of the underlying disease is crucial for preventing recurrence.