Rare Case of Acute Myocarditis Complicating Macrophagic Activation Syndrome: A Complex Clinical Conundrum
Published: 2024-06-21
Page: 79-86
Issue: 2024 - Volume 7 [Issue 1]
Anass El Mokri El Mghari *
Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco.
Afaf Elmouhdi
Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco.
Meriem Tabat
Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco.
Mohammed Megzari
Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco.
Abdessamad Asklou
Department of Cardiology, Ibn Rochd University Hospital, Casablanca, Morocco.
Salim Arous
Faculty of Medicine and Pharmacy of Casablanca, Morocco.
Abdenasser Drighil
Faculty of Medicine and Pharmacy of Casablanca, Morocco.
Rachida Habbal
Faculty of Medicine and Pharmacy of Casablanca, Morocco.
*Author to whom correspondence should be addressed.
Abstract
Macrophagic Activation Syndrome (MAS) and myocarditis individually pose significant challenges to healthcare providers due to their complex pathophysiology and diverse clinical presentations. However, when these two conditions intersect, the immune system's dysregulation amplifies the inflammatory response, posing unique diagnostic and therapeutic dilemmas. The clinical presentation is nonspecific, showing no pathognomonic signs, the patient may present chest pain, shortness of breath, fever and signs of systemic inflammation, which calls for heightened awareness and a comprehensive approach to make the diagnosis and for better management.
In the following paper, we report the case of a 23 years old man admitted to our hospital for dyspnea, and chest pain, associated to an altered general condition and fever, and in whom, a constellation of clinical features and laboratory findings, met the diagnostic criteria for MAS associated to a myocarditis. He was treated with high dose intravenous corticosteroid, and heart failure drugs, resulting in resolution of fever and dramatic clinical improvement.
Keywords: Hyperferritinemia, cytokine release storm, corticosteroid, myocarditis, macrophage activation syndrome (MAS)
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References
Créput C, Galicier L, Oksenhendler E, Azoulay E. Syndrome d'activation lymphohistiocytaire: Revue de la littérature, implications en réanimation. Réanimation. 2005;14(7):604–613. French
Scott RB, Robb-Smith HT. Histiocytic medullary reticulosis. Lancet. 1939;234 (6047):171–236.
Agarwal, Arun, Aakanksha Agarwal, Abu Saad Khan. Hemophagocytic lymphohistiocytosis – unusual presentation as cholangitis- cholestatic jaundice. Asian Journal of Medicine and Health. 2018;11(4):1-10. Available:https://doi.org/10.9734/ajmah/2018/41435.
Letsas KP, Filippatos GS, Delimpasi S, Spanakis N, Kounas SP, Efremidis M, Tsakris A, kardaras f. Enterovirus-induced fulminant myocarditis and hemophagocytic syndrome. Journal of Infection. 2007; 54(2):e75-7
Rosário C, zandman-goddard G, meyron-holtz EG, D’cruz DP, Shoenfeld Y. The hyperferritinemic syndrome: Macrophage activation syndrome, still’s disease, septic shock and catastrophic antiphospholipid syndrome. BMC Medicine. 11:1-1
Flechaire A, Colle B, Bernard P, Dupuy O, et al. Les syndromes hémophagocytaires. Rev Méd Interne. 1996;17(2):157–162.
Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome (HPS). Autoimmun Rev 2004;3:69—75.
Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C. Infections associated with haemophagocytic syndrome. Lancet Infect Dis 2007;7:814—22.
Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis: The FHL study group of the Histiocyte society. Semin Oncol. 1991;18(1):29–33.
Henter JI, Elinder G, Soder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand. 1991;80(4):428–435.
Grom, A., Horne, A. & De Benedetti, F. Macrophage activation syndrome in the era of biologic therapy. Nat Rev Rheumatol. 2016;12:259–268. Available:https://doi.org/10.1038/nrrheum.2015.179
Kumar A, Thota V, Dee L, Olson J, Uretz E, Parrillo JE. Tumor necrosis factor alpha and interleukin 1beta are responsible for in vitro myocardial cell depression induced by human septic shock serum. J Exp Med. 1 mars 1996;183(3):949 58.
Feldman AM, Combes A, Wagner D, Kadakomi T, Kubota T, Li YY, et al. The role of tumor necrosis factor in the pathophysiology of heart failure. J Am Coll Cardiol. 2000;35(3):537 44.
Vincent JL, Bakker J, Marécaux G, Schandene L, Kahn RJ, Dupont E. Administration of anti-TNF antibody improves left ventricular function in septic shock patients. Results of a pilot study. Chest. 1992;101(3):810 5.
Cooper N, Rao K, Gilmour K, Hadad L, Adams S, Cale C, et al. Stem cell transplantation with reduced-intensity conditioning for hemophagocytic lymphohistiocytosis. Blood. 2006;107(3): 1233 6.
Suková M, Starý J, Housková J, Nohýnková E. [Hemophagocytic lymphohistiocytosis as a manifestation of visceral leishmaniasis]. Cas Lek Cesk. 2002;141(18):581 4.
Schecter JP, Jones SE, Jackson RA. Myocardial infarction in a 27-year-old woman: possible complication of treatemtn with VP-16-213 (NSC-141540), mediastinal irradiation, or both. Cancer Chemother Rep. 1975;59(5):887 8.
Bergelson JM, Cunningham JA, Droguett G, Kurt-Jones EA, Krithivas A, Hong JS, et al. Isolation of a common receptor for Coxsackie B viruses and adenoviruses 2 and 5. Science. 1997;275(5304): 1320 3.
Vieillard-Baron A, Loubieres Y, Schmitt JM, Page B, Dubourg O, Jardin F. Cyclic changes in right ventricular output impedance during mechanical ventilation. J Appl Physiol Bethesda Md. 1999;87(5):1644 50.
Escoto H, Ringewald J, Kalpatthi R. Etoposide-related cardiotoxicity in a child with haemophagocytic lymphohistiocytosis. Cardiol Young. 2010;20(1):105 7.
Airey CL, Dodwell DJ, Joffe JK, Jones WG. Etoposide-related myocardial infarction. Clin Oncol R Coll Radiol GB. 1995;7 (2):135.
George MR. Hemophagocytic lymphohistiocytosis: Review of etiologies and management. J Blood Med. 2014;5:69 86.
Johnson TS, Terrell CE, Millen SH, Katz JD, Hildeman DA, Jordan MB. Etoposide selectively ablates activated T cells to control the immunoregulatory disorder hemophagocytic lymphohistiocytosis. J Immunol Baltim Md 1950. 2014;192 (1):84 91.
Imashuku S, Kuriyama K, Teramura T, Ishii E, Kinugawa N, Kato M, et al. Requirement for etoposide in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. J Clin Oncol Off J Am Soc Clin Oncol. 2001;19(10): 2665 73.
Chellapandian D, Das R, Zelley K, Wiener SJ, Zhao H, Teachey DT, et al. Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemoimmuno-therapeutic regimens. Br J Haematol. 2013; 162(3):376 82.
Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatol Oxf Engl. 2008;47(11):1686 91.
Das R, Guan P, Sprague L, Verbist K, Tedrick P, An QA, et al. Janus kinase inhibition lessens inflammation and ameliorates disease in murine models of hemophagocytic lymphohistiocytosis. Blood. 2016;127(13):1666 75.
Ahmed A, Merrill SA, Alsawah F, Bockenstedt P, Campagnaro E, Devata S, et al. Ruxolitinib in adult patients with secondary haemophagocytic lymphohistiocytosis: an open-label, single-centre, pilot trial. Lancet Haematol. 2019;6(12):e630 7.
Tschöpe, Carsten et al. Management of myocarditis-related cardiomyopathy in adults. Circulation Research. 2019;124 (11):1568-1583.