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Review
. 2024 Jan 2;22(1):4.
doi: 10.1186/s12969-023-00944-2.

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

Lian Zhang  1 , Yanwen Wei  1 , Ningjing Zeng  1 , Lianyu Wang  2 , Xinying Chen  2 , Jinghua Yang  2   3 , Xiaolan Xiao  4
Affiliations
Review

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

Lian Zhang et al. Pediatr Rheumatol Online J. .

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.

Keywords: Coronary artery involvement; Macrophage activation syndrome (MAS); Systemic juvenile idiopathic arthritis (sJIA).

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Conflict of interest statement

The authors declare that they have no competing interests. No conflicts of interest to disclose. The authors have no financial relationships relevant to this article to disclose.

Figures

Fig. 1
Fig. 1
Treatment Process Diagram
Fig. 2
Fig. 2
Time Distribution of Coronary Injury
See this image and copyright information in PMC

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References

    1. Martini A, Ravelli A, Avcin T, et al. Toward new classification criteria for juvenile idiopathic arthritis: first steps, pediatric rheumatology international trials organization international consensus. J Rheumatol. 2019;46(2):190–197. doi: 10.3899/jrheum.180168. - DOI - PubMed
    1. Calvo-Río V, Santos-Gómez M, Calvo I, et al. Anti-Interleukin-6 receptor Tocilizumab for severe juvenile idiopathic arthritis-associated uveitis refractory to anti-tumor necrosis factor therapy: a multicenter study of twenty-five patients. Arthritis Rheum. 2017;69(3):668–675. doi: 10.1002/art.39940. - DOI - PubMed
    1. Barut K, Adrovic A, Şahin S, Kasapçopur Ö. Juvenile idiopathic arthritis. Balkan Med J. 2017;34(2):90–101. doi: 10.4274/balkanmedj.2017.0111. - DOI - PMC - PubMed
    1. Koca B, Sahin S, Adrovic A, Barut K, Kasapcopur O. Cardiac involvement in juvenile idiopathic arthritis. Rheumatol Int. 2017;37(1):137–142. doi: 10.1007/s00296-016-3534-z. - DOI - PubMed
    1. Goldenberg J, Ferraz MB, Pessoa AP, et al. Symptomatic cardiac involvement in juvenile rheumatoid arthritis. Int J Cardiol. 1992;34(1):57–62. doi: 10.1016/0167-5273(92)90082-e. - DOI - PubMed

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