Perimyocarditis as first sign of systemic onset juvenile idiopathic arthritis treated successfully with anakinra: a case-based review

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Silvia Ciancia
Michela Cappella
Alessandro De Fanti
Lorenzo Iughetti


Arthritis, Idiopathic, Juvenile, Myocarditis, Perimyocarditis, Interleukin, Anakinra


Background: The involvement of myocardium and pericardium at the same time is very uncommon as first manifestation of juvenile idiopathic arthritis with systemic onset (soJIA).

Case: A fourteen years-old boy, referred with symptoms of acute gastroenteritis, developed a perimyocarditis as first manifestation of  Still’s Disease, after only one day from the admission. The rheumatologic disease was not responding to glucocorticoid treatment. The use of anakinra was the key point of the therapy and after its administration the patient started to recover fastly.

Conclusions: This case report describes cardiac involvement as first sign of soJIA and the successful use of anakinra inducing remission of soJIA not-responding to steroid therapy.


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1. Shenoi S., Wallace C.A. Diagnosis and Treatment of Systemic Juvenile Idiopathic Arthritis. J. of Pediatrics Jun 16, 2016
2. Petty R.E. et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004;31(2):390
3. Cimaz R. Systemic-onset of juvenile idiopathic arthritis. Autoimmunity Reviews 15(2016)931–934
4. Barnes M.G. et al. Subtype-specific peripheral blood gene expression profiles in recent onset juvenile idiopathic arthritis. Arthritis Rheum 2009;60:210212.
5. Ishikawa S. et al. Abnormal expression of the genes involved in cytokine networks and mitochondrial function in systemic juvenile idiopathic arthritis identified by DNA microarray analysis. Ann Rheum Dis 2009;68:264-72.
6. Ogilvie E.M., Khan A., Hubank M., Kellam P., Woo P. Specific gene expression profiles in systemic juvenile idiopathic arthritis. Arthritis Rheum 2007;56:1954-65.
7. Mondal R., Sarkar S., Ghoshal A., Sabui T.. Congestive Heart Failure: An Uncommon Presentation of Systemic Onset Juvenile Idiopathic Arthritis (SOJIA). Indian J Pediatr (January 2013) 80(1):67–69
8. Ghosh J. B. Juvenile Idiopathic Arthritis-Its Rare Presentation. Indian J Pediatr (February 2012) 79(2):262–264
9. Svantesson H., Akesson A., Eberhardt K., Elborgh R. Prognosis in juvenile rheumatoid arthritis with systemic onset. A follow-up study. Scand J Rheumatol. 1983;12(2):139-44.
10. Svantesson H., Björkhem G., Elborgh R. Cardiac involvement in juvenile rheumatoid arthritis. A follow-up study. Acta Paediatr Scand. 1983 May;72(3):345-50.
11. Miller J.J. 3rd, French J.W. Myocarditis in juvenile rheumatoid arthritis. Am J Dis Child. 1977 Feb;131(2):205-9.
12. Goldenberg J.1., Ferraz M.B., Pessoa A.P., Fonseca A.S., Carvalho A.C., Hilario M.O., Atra E. Symptomatic cardiac involvement in juvenile rheumatoid arthritis. Int J Cardiol. 1992 Jan;34(1):57-62.
13. Cottafava F., Cosso D., Guglieri F., Siri F., Tosca P., Morreale G., Lombardi J. Cardiac involvement in juvenile rheumatoid arthritis. Minerva Pediatr. 1991 Jul-Aug;43(7-8):505-9. Italian.
14. Ward S.C., Wiselka M.J., Nicholson K.G. Still's disease and myocarditis associated with recent mumps infection. Postgrad Med J. 1988 Sep;64(755):693-5.
15. Zeft A.S., Menon S.C., Miller D. Fatal myocarditis in a child with systemic onset juvenile idiopathic arthritis during treatment with an interleukin 1 receptor antagonist. Pediatr Rheumatol Online J. 2012 Apr 10;10:8.
16. Koca B., Sahin S., Adrovic A., Barut K., Kasapcopur O. Cardiac involvement in juvenile idiopathic arthritis. Rheumatol Int. 2017 Jan;37(1):137-142. Epub 2016 Jul 14. Review.
17. Ringold S. et al. 2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for the Medical Therapy of Children With Systemic Juvenile Idiopathic Arthritis and Tuberculosis Screening Among C. Arthritis and rheumatism. 2013;65:2499-2512.
18. Nigrovic PA. Review: is there a window of opportunity for treatment of systemic juvenile idiopathic arthritis? Arthritis Rheumatol 2014;66:140513.
19. Giancane G., Minoia F., Davì S., Bracciolini G., Consolaro A. and Ravelli A. IL-1 Inhibition in Systemic Juvenile Idiopathic Arthritis. Front. Pharmacol., 2016; 7:467
20. Piel-Julian M.L. et al. Early use of anakinra in adult-onset Still's disease myocarditis. Scand J Rheumatol. 2018 Nov;47(6):511-512. Epub 2018 Jan 2.
21. Choi A.D., Moles V., Fuisz A., Weissman G. Cardiac magnetic resonance in myocarditis from adult onset Still’s disease successfully treated with anakinra. Int J Cardiol (2014)172(1):e225–e227.
22. Oehler E., Valour F., Hachulla E., Ghawche F. Myopericarditis as the presenting manifestation of adult Still’s disease. Rev Med Interne 2014, 35(12):827–830.
23. Luconi N. et al. Myocarditis in a young man with adult onset Still's disease successfully treated with Il-1 blocker. Int J Cardiol. 2015;189:220-2. Epub 2015 Apr 11. Review.

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