Macrophage activation syndrome: an under-recognised complication in juvenile systemic lupus erythematosus

Macrophage activation syndrome (MAS) is a life-threatening complication in juvenile systemic lupus erythematosus(J SLE). We report three cases of MAS in Juvenile SLE which occurred over the last 6 months. Interestingly, in all the 3 cases, MAS occurred acutely at the time of first presentation of lupus. All of them had fever at presentation. Two of them had lymphadenopathy and splenomegaly and one had hepatomegaly. All three of them had renal, one had heart and lung and another had central nervous system involvement. Table 1 summarises the laboratory findings.

Table 1 Laboratory findings

Interestingly, the ferritin levels were <2000 in two of the three cases. In all the three cases, JSLE/MAS was suspected early and serology and bone marrow were done within 48 hours of referral to the rheumatologist. All three of them resolved with intravenous Methylprednisolone. Diagnosis of MAS can be difficult because some of its clinical features overlap those of lupus itself. High index of suspicion will help in early diagnosis and prompt initiation of treatment which are important for a better outcome.