MANAGEMENT OF MACROPHAGE ACTIVATION SYNDROME AND CONDURRENT LIFE-THREATENING INFECTION IN A PATIENT WITH JUVENILE IDIOPATHIC ARTHRITIS

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2013-04-12

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Sulak, Kyle

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Purpose: This report will discuss the manifestations and treatment of the rare complication of Macrophage Activation Syndrome (MAS) and superimposed life-threatening Cytomegalovirus (CMV) infection in Juvenile Idiopathic Arthritis (JIA). We will also discuss the potential causative factors of MAS. Methods: This report will discuss the manifestations and treatment of the rare complication of Macrophage Activation Syndrome (MAS) and superimposed life-threatening Cytomegalovirus (CMV) infection in Juvenile Idiopathic Arthritis (JIA). We will also discuss the potential causative factors of MAS. Results: Current treatment recommendations indicate management of JIA with Anakinra, low dose Prednisolone and Methotrexate. Indications that point to the diagnosis of MAS include an elevated PT, thrombocytopenia, hypofibrinogenemia, elevated D-dimers, elevated aspartate aminotransferase and clinical presentation. Clinical recommendations for the treatment of MAS include fresh frozen plasma, corticosteroids and supportive care as well as Cyclosporine when patients do not respond to those treatments. Initial treatment for CMV is intravenous ganciclovir. Additionally, immune globulin may be administered as well as foscarnet and vanganciclovir. Previous studies point to inciting agents of MAS as well as lab criteria for distinguishing the onset of MAS from an increase in systemic symptoms of JIA. Conclusions: In this patient's case, the onset of MAS is likely multifactorial and may include both CMV infection and use of methotrexate. This case showed that management of autoimmune disorders with life-threatening infections is challenging as treatment for one condition may worsen the other. Also, when evaluating laboratory data, it is important to remember that JIA causes an increase in many inflammatory markers, so a relative decrease may be more indicative of MAS than absolute low values. This may warrant for routine monitoring of inflammatory markers in order to make comparisons if a patient's symptoms worsen and MAS is suspected.

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