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A rare case of coronary artery complication in a child with systemic juvenile idiopathic arthritis and macrophage activation syndrome: case report and literature review
Pediatric Rheumatology ( IF 2.5 ) Pub Date : 2024-01-02 , DOI: 10.1186/s12969-023-00944-2
Lian Zhang , Yanwen Wei , Ningjing Zeng , Lianyu Wang , Xinying Chen , Jinghua Yang , Xiaolan Xiao

A rare case of coronary artery involvement in a child with Systemic Juvenile Idiopathic Arthritis (sJIA) complicated by Macrophage Activation Syndrome (MAS) is reported. The patient initially received an inaccurate diagnosis of Kawasaki Disease, sepsis, and mycoplasma infection and showed no improvement after Intravenous Immune Globulin (IVIG) treatment. Upon admission, symptoms included diffuse red rash, swelling of the limbs, lymph node enlargement, and hepatosplenomegaly. Post investigations, a diagnosis of sJIA and MAS was confirmed, and treatment involved a combination of hormones (methylprednisolone) and immunosuppressive drugs (methotrexate). The revealed widened coronary artery diameter was managed with a disease-specific treatment plan and prophylactic plus low-dose aspirin anti-coagulation therapy. Under this management, MAS was well controlled, and follow-ups showed normalization of the child’s coronary artery structure and function. This case and the associated literature review underscore the importance of early recognition, diagnosis, treatment, and long-term monitoring for children presenting with sJIA and MAS complicated by coronary artery involvement.

中文翻译:

全身性幼年特发性关节炎合并巨噬细胞活化综合征患儿冠状动脉并发症一例罕见病例报告及文献复习

据报道,患有系统性幼年特发性关节炎 (sJIA) 并发巨噬细胞激活综合征 (MAS) 的儿童冠状动脉受累的罕见病例。该患者最初被错误诊断为川崎病、败血症和支原体感染,经过静脉注射免疫球蛋白(IVIG)治疗后没有任何改善。入院时症状包括弥漫性红疹、四肢肿胀、淋巴结肿大、肝脾肿大。经过调查,确诊为 sJIA 和 MAS,并采用激素(甲泼尼龙)和免疫抑制药物(甲氨蝶呤)联合治疗。通过针对疾病的治疗计划和预防性加低剂量阿司匹林抗凝治疗来治疗所发现的冠状动脉直径增宽。在这种管理下,MAS得到了很好的控制,随访显示孩子的冠状动脉结构和功能正常化。该病例和相关文献综述强调了对患有 sJIA 和 MAS 并发冠状动脉受累的儿童进行早期识别、诊断、治疗和长期监测的重要性。
更新日期:2024-01-02
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