Macrophage activation syndrome (MAS) in juvenile systemic lupus erythematosus (JSLE): an underrecognized complication?

Objective

To define the characteristics of MAS complicating JSLE.

Methods

Patients with JSLE and MAS were collected from: 1) Gaslini Institute of Genoa, Italy; 2) PRINTO and PRCSG investigators; 3) literature. Control groups of JSLE without MAS included 33 patients with active lupus seen at Gaslini Institute (SLE-GI) and 387 patients from a multinational study of damage in JSLE (SLE-MS). Clinical and laboratory features of MAS with (BM+) or without (BM-) bone marrow demonstration of haemophagocytosis were contrasted each other and with those of lupus without MAS.

Results

20 BM+ and 18 BM-patients with JSLE-associated MAS were identified. Comparison of percentage frequency of the main clinical and laboratory features of MAS in patient groups is shown in table 1.

Table 1 Comparison of percentage frequency of the main clinical and laboratory features of MAS in partient groups. (NA: not available)

Conclusion

Features of MAS in patients with or without BM haemophagocytosis were comparable, except for a greater frequency of leukopenia in BM+ patients. This suggests that this complication is more common than previously realized. All features but leukopenia and fever discriminated well between MAS and active lupus without MAS.