INTRODUCTION: Macrophage activation syndrome (MAS) is a life-threatening 
hyperinflammatory state mediated by uncontrolled cytokine storm and 
haemophagocytosis. Although rarely reported, MAS might occur in systemic lupus 
erythematosus (SLE), notably as an inaugural manifestation. Glucocorticoids 
(GCs) are the cornerstone of SLE therapy. However, in some cases high doses of 
GCs are required to achieve remission (i.e. glucocorticoid-resistance), leading 
to significant side effects.
CASE REPORT: A 28-year-old Romani male was admitted to our hospital for 
polyarthralgia, polyserositis and fatigability. The patient had high-grade 
fever, jaundice and generalized lymphadenopathy. Laboratory tests revealed 
severe mixed hemolytic autoimmune anemia, leukopenia, hepatocytolysis, 
coagulation abnormalities, hypertriglyceridemia, biological inflammatory 
syndrome, hyperferritinemia and persistent proteinuria of nephritic pattern. 
Imaging studies showed pleuropericardial effusion, hepatosplenomegaly and 
polysynovitis. Additional blood tests revealed hypocomplementemia and positive 
ANA, anti-dsDNA and anti-Sm antibodies. Haemophagocytosis was not identified 
either on bone marrow or axillary lymph node biopsy specimens. However, 
SLE-associated MAS seemed to fit this set-up. High-dose corticotherapy (6.5 g 
methylprednisolone followed by prednisone, 1.5 mg/kg/day after discharge) and 
intravenous cyclophosphamide were necessary to induce and sustain remission.
CONCLUSION: MAS is a potentially severe manifestation that should be considered 
at SLE onset whenever high fever and elevated serum levels of aspartate 
aminotransferase, lactate dehydrogenase, C-reactive protein, ferritin and 
procalcitonin are noted. Early diagnosis and prompt treatment lead to remission 
in two thirds of cases. Glucocorticoid-resistance leads to the use of high-dose 
corticotherapy or immunosuppressive agents that could elicit serious side 
effects. New insights into the molecular mechanisms of glucocorticoid-resistance 
are needed in order to conceive more adequate GC-therapies.