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)
| MAS BM+ | MAS BM- | SLE-GI | SLE-MS | |
|---|---|---|---|---|
| Fever | 95.0 | 83.3 | 21.2 | 64.2 |
| Hepatomegaly | 47.4 | 55.6 | 12.1 | 10.4 |
| CNS dysfunction | 37.5 | 28.6 | 3.0 | 8.5 |
| Haemorrhages | 40.0 | 33.3 | 9.1 | NA |
| Leukopenia | 90.0 | 44.4 | 63.6 | NA |
| Thrombocytopenia | 90.0 | 61.1 | 18.2 | NA |
| Hypertransaminasemia | 80.0 | 93.8 | 30.3 | NA |
| Hypertriglyceridemia | 75.0 | 88.2 | 20.0 | NA |
| Hypofibrinogenemia | 37.5 | 42.9 | 0 | NA |
| Hyperferiitinemia | 92.9 | 94.4 | 0 | NA |
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.