Clinical manifestations of AOSD patients with and without MAS
| AOSD with MAS (60) | AOSD without MAS (390) | P value | |
| Sex (male/female） | 18/42 | 72/318 | 0.055 |
| Age (years) | 29 (22-37) | 38 (27-50) | <0.0001 |
| Death (n, %) | 13 (21.67%) | 8 (2.05%) | <0.0001 |
| Fever (n, %) | 60 (100%) | 255 (65.38%) | <0.0001 |
| Active arthritis (n, %) | 19 (31.67%) | 372 (95.38%) | <0.0001 |
| Splenomegaly | 50 (83.33%) | 52 (13.33%) | <0.0001 |
| Central nervous system disease | 1 (1.67%) | 0 | 0.133 |
| Haemorrhagic manifestations | 1 (1.67%) | 3 (0.77%) | 0.437 |
| Platelet count (×109/L) | 90 (60-144) | 244 (222-485) | <0.0001 |
| Liver dysfunction (n, %) | 58 (96.67%) | 73 (18.72%) | <0.0001 |
| Lactic dehydrogenase (U/L) | 1024 (599–2145) | 313 (222-485) | <0.0001 |
| Triglycerides (mmol/L) | 2.35 (1.82–3.78) | 1.42 (0.97–2.03) | <0.0001 |
| Fibrinogen (mg/dL) | 151 (104-219) | 306 (52-452) | <0.0001 |
| Ferritin (ng/mL)* | 1500 (1500–1500) | 1264 (359–1500) | <0.0001 |
| Bone marrow hemophagocytosis (n, %) | 39 (65%) | 13 (3.33%) | <0.0001 |
| MS score (median) | −0.01 (-0.27 to 0.50) | −2.67 (-3.51 to 1.82) | <0.0001 |
*The up limit of the detection of ferritin was 1500 ng/mL in our centres.
AOSD, adult-onset Still’s disease; MAS, macrophage activation syndrome.