Case Reports

Adult-onset Still’s disease with secondary macrophage activation syndrome: a case report

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Received: 20 April 2023
Published: 29 May 2023
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We describe a case of adult-onset Still’s disease (AOSD) with secondary macrophage activation syndrome. AOSD is a rare disease with unknown origin. Rarely it’s complicated by secondary macrophage activation syndrome, which is a life-threatening disorder. Because the diagnosis of AOSD is essential for exclusion and complications are life-threatening, it is mandatory for a prompt and extensive diagnostic workup and treatment.

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Magadur-Joly G, Billaud E, Barrier JH, et al. Epidemiology of adult Still’s disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis 1995;54:587-90. DOI: https://doi.org/10.1136/ard.54.7.587

Castaneda S, Blanco R, Gonzalez-Gay MA. Adult-onset Still’s disease: advances in treatment. Best Pract Res Clin Rheumatol 2016;30:222-38. DOI: https://doi.org/10.1016/j.berh.2016.08.003

Efthimiou P, Kontzias A, Hur P, et al. Adult-onset Still’s disease in focus: clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum 2021;51:858-74. DOI: https://doi.org/10.1016/j.semarthrit.2021.06.004

Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992;19:424-30.

Al-Samkari H, Berliner N. Hemophagocytic Lymphohistiocytosis. Annu Rev Pathol Mech Dis 2018;13:27-49. DOI: https://doi.org/10.1146/annurev-pathol-020117-043625

Crayne CB, Albeituni S, Nichols KE, et al. The immunology of macrophage activation syndrome. Front Immunol 2019:10:119. DOI: https://doi.org/10.3389/fimmu.2019.00119

Jordan MB, Hildeman D, Kappler J and Marrack P. An animal model of hemophagocitic lymphoistiocytosis (HLH); CD8 T cells and interferon gamma are essential for the disorder. Blood 2004;104:735-43. DOI: https://doi.org/10.1182/blood-2003-10-3413

Schulert GS, Cron RQ. The genetics of macrophage activation syndrome. Genes Immun 2020;21:169-81. DOI: https://doi.org/10.1038/s41435-020-0098-4

Kaufman KM, Linghu B, Szustakowski JD, et al. Wholeexome sequencing reveals overlap between macrophage activation syndrome in systemic juvenile idiopathic arthritis and familial hemophagocytic lymphohistiocytosis. Arthritis Rheumatol 2014;66:3486-95. DOI: https://doi.org/10.1002/art.38793

Dhore R, Simon J, Papo T, et al. Reactive hemophagocitic syndrome adult systemic disease: report of twenty-six cases and literature review. Arthritis Rheum 2003;49:633-9. DOI: https://doi.org/10.1002/art.11368

Fukaya S, Yasuda S, Hashimoto T, et al. Clinical features of hemophagocytic syndrome in patients with systemic autoimmune disease: analysis of 30 cases. Rheumatology (Oxford) 2008;47:1686-91. DOI: https://doi.org/10.1093/rheumatology/ken342

Yang XP, Wang M., Li TF, et al. Predictive factors and prognosis of macrophage activation syndrome associated with adult-onset Still’s disease. Clin Expe Rheumatol 2019:37:83-8.

Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive

hemophagocytic syndrome. Arthritis Rheumatol 2014;66:2613-20. DOI: https://doi.org/10.1002/art.38690

Henter JI, Horne A, Arico’ M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-31. DOI: https://doi.org/10.1002/pbc.21039

Fajgenbaum D, June C. Cytokine Storm. NEJM 2020; 383:2255-73. DOI: https://doi.org/10.1056/NEJMra2026131

Giuseppe Nicoletti, Internal Medicine, Madonna delle Grazie Hospital, Matera

Internal Medicine director

Antonio Bonelli, Internal Medicine, Madonna delle Grazie Hospital, Matera

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Rocco Clemente, Internal Medicine, Madonna delle Grazie Hospital, Matera

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Simona Ciuffreda, Internal Medicine, Madonna delle Grazie Hospital, Matera

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Gaetano Dentamaro, Internal Medicine, Madonna delle Grazie Hospital, Matera

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Agata Tiziana La Masa, Internal Medicine, Madonna delle Grazie Hospital, Matera

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Vito Lascaro, Internal Medicine, Madonna delle Grazie Hospital, Matera

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Pasquale Santarcangelo, Internal Medicine, Madonna delle Grazie Hospital, Matera

MD

How to Cite



Adult-onset Still’s disease with secondary macrophage activation syndrome: a case report. (2023). Italian Journal of Medicine, 17(1). https://doi.org/10.4081/itjm.2023.1595