Macrophage activation syndrome in the course of adult-onset Still’s disease – a case report

Marzena Lewandowska, Marek Brzosko

Abstract


Introduction: Macrophage activation syndrome (MAS) is a rare complication of autoimmune diseases. The background of the diseases is immune system dysfunction. The disease is characterized by varying symptomatology and the different dynamics of accumulation of symptoms.

Case report: A female patient diagnosed with adult-onset Still’s disease was admitted to the hospital because of fever, weakness, skin rash and dyspepsia which had lasted for several days. Diagnostic tests revealed the presence of anti-nuclear antibodies, pancytopenia, hypertriglyceridemia, hyperferritinemia, hypofibrinogenemia, elevated activity of liver enzymes, and increased levels of C-reactive protein. The initial treatment with empirical antibiotic therapy and intravenous pulses of methylprednisolone was unsuccessful and provided no improvement. On the basis of bone marrow cytological examination, the diagnosis of MAS was established. The patient was treated with dexamethasone and cyclosporine A with improvement. The patient was followed up in the rheumatology out-patient department for 20 months without the relapse of MAS.

Conclusion: Macrophage activation syndrome is a rare, dangerous complication of autoimmune diseases, requiring rapid diagnosis and aggressive treatment.


Keywords


secondary haemophagocytic syndrome; haemophagocytic lymphohistiocytosis; adult-onset Still’s diseases; dexamethasone; cyclosporine

Full Text:

PDF (Język Polski)

References


Ziętkiewicz M, Hajduk A, Wojtaczek A, Smoleńska Ż, Czuszyńska Z, Zdrojewski Z. Zespół aktywacji makrofagów – opis trzech przypadków. Ann Acad Med Stetin 2012;58(1):55-61.

Deane S, Selmi C, Teuber SS, Gershwin ME. Macrophage activation syndrome in autoimmune disease. Int Arch Allergy Immunol 2010;153(2): 109-20. doi: 10.1159/000312628.

Zoń-Giebel A, Giebel S. Zespół aktywacji makrofaga – reaktywna postać limfohistiocytozy hemofagocytarnej. Reumatologia 2008;46:21-6.

Kelly A, Ramanan AV. Recognition and menagment of macrophage activation syndrome in juvenile arthritis. Curr Opin Rheumatol 2007;19: 477-81.

Henter J-I. HLH-2004 protocol [Internet]. Histiocyte Society 2004.

Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr 2007;166:95-109.

Filipovich A. Hemophagocytic lymphohistiocytosis and rother hemophagocytic disorders. Immunol Allergy Clin North Am 2008;28:293-313.

Areci R. When T cells and macrophages do not talk: hehemophagocytic syndromes. Curr Opin Hematol 2008;15:359-67.

Buda P, Gietka P, Wieteska-Klimczak A, Książyk J. Wtórne zespoły hemo­fagocytarne – aktualny problem interdyscyplinarny. Wiad Lek 2013;66(2): 153-63.

Rojek-Margas B, Śliwowska B, Bucka J. Zespół aktywacji makrofaga. Reumatologia 2013;51(6):459-66.

Szczeklik A, Gajewski P. Interna Szczeklika 2014.Podręcznik chorób wewnętrznych. Kraków: Medycyna Praktyczna; 2014. p. 1722-4.

Klaudel-Dreszler M, Rutynowska-Pronicka O, Gietka P, Rokicki D, Dembowska-Bagińska B, Piątosa B, et al.

Propozycja standardu diagnostyczno-terapeutycznego dla dzieci z podejrzeniem pierwotnego lub wtórnego zespołu hemofagocytarnego w oparciu o doświadczenia Instytutu „Pomnik – Centrum Zdrowia Dziecka” w Warszawie. Stand Med 2010;7:194-205.

Tristano AG. Macrophage activation syndrome: a frequent but under-diagnosed complication associated with rheumatic diseases. Med Sci Monit 2008;14:27-36.

Ravelli A. Macrophage activation syndrome. MD. Pediatria II. Curr Opin Rheumatol 2002;14:548-552.

Takahashi K, Kumakura S, Ishikura H, Murakawa Y, Yamauchin Y, Kobayashi S. Reactive hemophagocytosis in systemic lapus erythematosus. Intern Med 1998;37(6):550-3.

Grom AA, Mellins ED. Macrophage activation syndrome: advances towards under standing pathogenesis. Curr Opin Rheumatol 2010;22:561-6.

Machaczka M. Specyfikacja występowania limfohistiocytozy hemofagocytarnej w okresie wieku dorosłego. Acta Haematol Pol 2013;44:307-13.

Hanter JI, Horne A, Arico M, Egeer RM, Filipovich AH, Imashuku S, et al.: HLH-2004: Diagnstic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-31.

Machaczka M, Klimkowska M. Limfohistiocytoza hemofagocytarna – kiedy nadmierna odpowiedź immunologiczna wymyka się mechanizmom kontrolnym. Przypadki Medyczne 2013;45:196-205.

Sawhney S, Woo P, Murray KJ. Macrophage activtion syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child 2001;85:421-6.

Lin C-I, Yu H-H, Lee J-H, Wang LC, Lin YT, Yang YH, et al. Clinical analysis of macrophage activation syndrome in pediatric patient with autoimmune disease. Clin Rheumatol 2012;31:1223-30.

Sen ES, Clarke SL, Ramanan AV. Macrophage activation syndrome. Indian J Pediatr 2016; 83(3):248-53. doi: 10.1007/s12098-015-1877-1.

Larroche C. Hemophagocytic lymphohistiocytosis in adults: Diagnosis and treatment. Joint Bone Spine 2012;79:356-61.

Pallazi DL, McClain KL, Kaplan SL. Hemophagocytic syndrome in children: an important diagnostic cnsideration in fever of unknow origin. Clin Infect Dis 2003;36:306-12.

Ravelli A, Grom AA, Behrens EM, Cron RQ. Macrophage activation syndrome as part of systemic juvenile idiophatic arthritis: diagnosis, genetics, pathophysiology, and treatment. Genes Immun 2012;13:289-98.

Atteritano M, David A, Bagnato G, Beninati C, Frisina A, Iaria C, et al. Haemophagocytic syndrome in rheumaticpatients. Systemie review. Eur Rev Med Pharmacol Sci 2012;16:1414-24.




DOI: https://doi.org/10.21164/pomjlifesci.232

Copyright (c) 2017 Marzena Lewandowska, Marek Brzosko Brzosko

License URL: https://creativecommons.org/licenses/by-nc-nd/3.0/pl/