Relapsing macrophage activating syndrome in a 15-year-old girl with Still's disease: a case report
IntroductionMacrophage activating syndrome (MAS) is a severe, potentially life-threatening condition which may accompany Still's disease (SD). It is characterized by fever, hepatosplenomegaly, lymphadenopathy, severe cytopenia, serious liver dysfunction, coagulopathy and neurologic involvement.
The principal treatment for patients with this syndrome includes etoposide 150mg/2M twice a week for 2 weeks, dexamethasone 10mg/2M for 2 weeks and cyclosporine 3-5 mg/kg for long period. Cases of relapse of MAS are relatively rare.Case report: We describe a patient with SD who developed macrophage activating syndrome with ARDS requiring resuscitation and mechanical ventilation.
Following intensive treatment, including high dose steroids and cyclosporine, she improved significantly but two weeks after the discontinuation of cyclosporine she was readmitted with an acute relapse of MAS manifested by spiking fever, arthralgias, maculopapular rash and leukocytosis. This time the patient recovered following renewal of the treatment with cyclosporine and addition of mofetil micophenolate (celcept).
Conclusions: We believe that cyclosporine is a cornerstone for treatment in these cases.
We recommend continuing this medication forseveral weeks following the patient's clinical recovery in order to prevent MAS relapses.
Author: Meir MizrahiEldad Ben-Chetrit
Credits/Source: Journal of Medical Case Reports 2009, 3:138
Published on: 2009-11-19
Copyright by the authors listed above - made available via BioMedCentral (Open Access). Please
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