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2017

Copyright (c) 2017 Ornella Ruiz, Carlos Gallón, Tatiana González

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Macrophagic activation syndrome as a complication of systemic juvenile idiopathic arthritis: A case report

https://doi.org/10.17081/innosa.4.2.2630
Ornella Ruiz
E.S.E. Hospital Niño Jesús de Barranquilla, Colombia
Carlos Gallón
Organización Clínica General del Norte. Barranquilla, Colombia.
Tatiana González
Organización Clínica General del Norte. Barranquilla, Colombia.

Corresponding Author(s) : Ornella Ruiz

ornella.ruiz@hotmail.com

Ciencia e Innovación en Salud, 2017

Abstract

Background: Macrophagic activation syndrome (MAS) may appear as a complication in patients with rheumatic diseases, it has been more frequently associated with the systemic form of juvenile idiopathic arthritis and systemic lupus erythematosus.
Case report: We present the case of a school-aged patient, who debuted with MAS as a complication of juvenile systemic idiopathic arthritis, refractory to conventional treatments and with an adequate response to biological therapy.
Discussion: MAS is a life-threatening autoinflammatory disorder, characterized by the activation of the intracellular inflamosoma with proliferation of T lymphocytes and the mononuclear phagocytic system, and hyperproduction of inflammatory prostaglandins such as IL 1, IL 6 and IL 18; its diagnosis is based on a combination of clinical signs and biochemical and histological abnormalities.

Keywords

Macrophage activation syndrome, inflammation, arthritis juvenile.
1.
Ruiz O, Gallón C, González T. Macrophagic activation syndrome as a complication of systemic juvenile idiopathic arthritis: A case report. Ciencia e Innovación en Salud [Internet]. 2017 Dec. 11 [cited 2024 Nov. 27];4(1). Available from: https://revistas.unisimon.edu.co/index.php/innovacionsalud/article/view/2630
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References
  • Aeberli D, Oertle S, Mauron H, Reichenbach S, Jordi B, Villiger PM. 2002. Inhibition of the TNF-pathway: use of infliximab and etanercept as remission-inducing agents in cases of therapy-resistant chronic inflammatory disorders. Swiss Med Wkly. 27;132(29-30):414-422. PubMed PMID: 12428187. Epub 2002/11/13. eng.
  • Athreya BH. 2002. Is macrophage activation syndrome a new entity? Clin Exp Rheumatology. Mar-Apr;20(2):121-123. PubMed PMID: 12051388. Epub 2002/06/08. eng.
  • Avcin T, Tse SM, Schneider R, Ngan B, Silverman ED. 2006. Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood. J Pediatr. 148(5):683- 686. PubMed PMID: 16737887. Epub 2006/06/02. eng.
  • Behrens EM, Beukelman T, Paessler M, Cron RQ. 2007. Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol. 34(5):1133- 1138. PubMed PMID: 17343315. Epub 2007/03/09. eng.
  • Coca A, Bundy KW, Marston B, Huggins J, Looney RJ. 2009. Macrophage activation syndrome: serological markers and treatment with anti-thymocyte globulin. Clinical immunology (Orlando, Fla).132(1):10-18. PubMed PMID: 19297252. Epub 2009/03/20. eng. DOI 10.1016/j.clim.2009.02.005
  • Cuende E, Vesga JC, Perez LB, Ardanaz MT, Guinea J. 2001. Macrophage activation syndrome as the initial manifestation of systemic onset juvenile idiopathic arthritis. Clin Exp Rheumatol. 19(6):764-765. PubMed PMID: 11791663. Epub 2002/01/17. eng.
  • Deane S, Selmi C, Teuber SS, Gershwin ME. 2010. Macrophage activation syndrome in autoimmune disease. Int Arch Allergy Immunol. 153(2):109-120. PubMed PMID: 20407267. Epub 2010/04/22. eng DOI 10.1159/000312628
  • Emmenegger U, Frey U, Reimers A, Fux C, Semela D, Cottagnoud P, Spaeth PJ, Neftel KA. 2001. Hyperferritinemia as indicator for intravenous immunoglobulin treatment in reactive macrophage activation syndromes. Am J Hematol. 68(1):4-10. PubMed PMID: 11559930. Epub 2001/09/18. eng.
  • Grom AA, Passo M. 1996. Macrophage activation syndrome in systemic juvenile rheumatoid arthritis. J Pediatr. 129(5):630-632. PubMed PMID: 8917224. Epub 1996/11/01. eng. DOI 10.1002/art.23734
  • Gupta A, Tyrrell P, Valani R, Benseler S, Weitzman S, Abdelhaleem M. 2008. The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. Sep;51(3):402-404. PubMed PMID: 18523990. Epub 2008/06/05. eng. DOI 10.1002/pbc.21564
  • Hadchouel M, Prieur AM, Griscelli C. 1985. Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: possible relationship to drugs or infection. J Pediatr. 106(4):561-566. PubMed PMID: 3981309. Epub 1985/04/01. eng.
  • Hone N, Donnelly C, Houk JB, Mina R. 2017. Macrophage Activation Syndrome in Scleroderma. Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases. J Clin Rheumatol. 23(2):120-121. PubMed PMID: 28225516. Epub 2017/02/23. eng. DOI 10.1097/RHU.0000000000000512
  • Hone N, Donnelly C, Houk JB, Mina R. 2017. Macrophage Activation Syndrome in Scleroderma. Mar;23(2):120-121. DOI 10.1097/RHU.0000000000000512
  • Janka GE. 1983. Familial hemophagocytic lymphohistiocytosis. Eur J Pediatr. 140(3):221-230. PubMed PMID: 6354720. Epub 1983/06/01. eng.
  • Kumakura S. 2005. Hemophagocytic syndrome. Intern Med. Apr; 44(4):278-280. PubMed PMID: 15897634. Epub 2005/05/18. eng.
  • Latino GA, Manlhiot C, Yeung RS, Chahal N, McCrindle BW. 2010. Macrophage activation syndrome in the acute phase of Kawasaki disease. J Pediatr Hematol Oncol. 32(7):527-531. PubMed PMID: 20485197. Epub 2010/05/21. eng. DOI 10.1097/MPH.0b013e3181dccbf4
  • Parodi A, Davi S, Pringe AB, Pistorio A, Ruperto N, Magni-Manzoni S, et al. 2009. Macrophage activation syndrome in juvenile systemic lupus erythematosus: a multinational multicenter study of thirty-eight patients. Arthritis Rheum. 60(11):3388-3399. PubMed PMID: 19877067. Epub 2009/10/31. eng. DOI 10.1002/art.24883
  • Ravelli A, Davi S, Minoia F, Martini A, Cron RQ. 2015. Macrophage Activation Syndrome. Hematol Oncol Clin North Am. 29(5):927-941. PubMed PMID: 26461152. Epub 2015/10/16. eng. DOI 10.1016/j.hoc.2015.06.010
  • Ravelli A, Grom AA, Behrens EM, Cron RQ. 2012. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immun. 13(4):289-298. PubMed PMID: 22418018. Epub 2012/03/16. eng. DOI 10.1038/gene.2012.3
  • Rossi-Semerano L, Hermeziu B, Fabre M, Kone-Paut I. 2011. Macrophage activation syndrome revealing familial Mediterranean fever. Arthritis Care Res (Hoboken). 63(5):780-783. PubMed PMID: 21557533. Epub 2011/05/11. eng. DOI 1002/acr.20418
  • Schulert GS & Grom AA. 2014. Macrophage activation syndrome and cytokine-directed therapies. Best Pract Res Clin Rheumatol. 28(2):277-292. PubMed PMID: 24974063. Pubmed Central PMCID: PMC4074772. Epub 2014/06/30. eng. DOI 10.1016/j.berh.2014.03.002
  • Schulert GS & Grom AA. 2015. Pathogenesis of macrophage activation syndrome and potential for cytokine- directed therapies. Annu Rev Med. 66:145-159. PubMed PMID: 25386930. Epub 2014/11/12. eng. DOI 10.1146/annurev-med-061813-012806
  • Stephan JL, Kone-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM. 2001. Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology (Oxford). Nov;40(11):1285-92. PubMed PMID: 11709613. Epub 2001/11/16. eng.
  • Usmani GN, Woda BA, Newburger PE. 2013. Advances in understanding the pathogenesis of HLH. Br J Haematol. 161(5):609-622. PubMed PMID: 23577835. Epub 2013/04/13. eng.
  • Wallace CA & Sherry DD. 1997. Trial of intravenous pulse cyclophosphamide and methylprednisolone in the treatment of severe systemic-onset juvenile rheumatoid arthritis. Arthritis and Rheum. 40(10):1852-1855. PubMed PMID: 9336421. Epub 1997/10/23. eng
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