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儿童全身型幼年特发性关节炎伴巨噬细胞活化综合征并发冠状动脉并发症罕见病例:病例报告及文献复习。

A rare case of coronary artery complication in a child with systemic juvenile idiopathic arthritis and macrophage activation syndrome: case report and literature review.

机构信息

The Second Clinical Medical College, Guangzhou University of Chinese Medicine, Guangzhou, China.

Department of Pediatrics, The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, China.

出版信息

Pediatr Rheumatol Online J. 2024 Jan 2;22(1):4. doi: 10.1186/s12969-023-00944-2.

Abstract

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.

摘要

本文报告了一例罕见的儿童全身型幼年特发性关节炎(sJIA)合并巨噬细胞活化综合征(MAS)累及冠状动脉的病例。该患儿最初被误诊为川崎病、脓毒症和支原体感染,静脉注射免疫球蛋白(IVIG)治疗后无改善。入院时,患儿表现为弥漫性红疹、四肢肿胀、淋巴结肿大和肝脾肿大。进一步检查后,确诊为 sJIA 和 MAS,治疗方案包括激素(甲泼尼龙)和免疫抑制剂(甲氨蝶呤)。发现冠状动脉扩张后,制定了针对该疾病的治疗方案,并进行了预防性和低剂量阿司匹林抗凝治疗。在这种治疗方案下,MAS 得到了很好的控制,随访显示患儿的冠状动脉结构和功能恢复正常。该病例及相关文献复习强调了对 sJIA 和 MAS 合并冠状动脉受累患儿进行早期识别、诊断、治疗和长期监测的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/69f2/10759671/1949f099d9ca/12969_2023_944_Fig1_HTML.jpg

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