Refractory macrophage activation syndrome in a patient with SLE and APLA syndrome - Successful use of PET- CT and Anakinra in its diagnosis and treatment

A patient with antiphospholipid syndrome, SLE and refractory fever is described. The cause for the fever was macrophage activation syndrome (MAS). The diagnosis of MAS was made with the help of PET-CT. Since the syndrome was refractory to conventional therapy with high-dose steroids and cyclosporin, anakinra was administered with complete recovery of the patient. The present case illustrates the difficulties in diagnosing MAS when multiple bone marrow biopsies fail to show hemophagocytosis. It emphasizes the significance of PET-CT in the diagnosis and evaluation of treatment of MAS. Finally, it describes the important role of Anakinra in treating refractory cases of MAS.