Comparison of clinical features between patients with and without MAS, at the time of diagnosis. Values are n (%) or mean ± SD unless otherwise specified.
| Characteristics | Without MAS | Presenting with MAS | p* |
|---|---|---|---|
| Women (men), n | 42 (65) | 6 (6) | 0.472 |
| Age, yrs | 44.8 ± 15.9 | 45.1 ± 17.7 | 0.677 |
| Clinical features | |||
| Fever | 107 (100) | 12 (100) | – |
| Arthritis | 94 (87.8) | 10 (83.3) | 0.655 |
| Myalgia | 64 (59.8) | 9 (75) | 0.306 |
| Rash | 81 (75.7) | 9 (75) | 0.957 |
| Splenomegaly | 74 (69.1) | 11 (91.6) | 0.102 |
| Liver involvement | 61 (57) | 11 (91.6) | 0.02 |
| Lymphadenopathy | 54 (50.4) | 10 (83.3) | 0.026 |
| Sore throat | 60 (56.1) | 10 (83.3) | 0.069 |
| Lung involvement | 13 (12.1) | 2 (16.6) | 0.655 |
| Pericarditis | 18 (16.8) | 3 (25) | 0.481 |
| Pleurisy | 15 (14.0) | 5 (41.7) | 0.083 |
| Abdominal pain | 16 (14.9) | 4 (33.3) | 0.106 |
| Weight loss | 2 (1.9) | 5 (41.7) | < 0.0001 |
| Systemic score, median (IQR) | 5 (4.0–7.0) | 8 (6.2–8.7) | 0.002 |
| Comorbidities | 32 (29.9) | 6 (50) | 0.330 |
| Laboratory markers | |||
| Leukocytosis > 15,000/mm3 | 49 (45.8) | 5 (41.7) | 0.785 |
| Serum ferritin, ng/ml, median (IQR) | 1120 (647.0–2393.5) | 3510 (1586.2–7444.0) | 0.001 |
| ESR, mm/h | 69.5 ± 25.9 | 74.5 ± 30.3 | 0.525 |
| CRP, mg/l, median (IQR) | 51 [19.7–119.0] | 45 [18.7–114.0] | 0.261 |
| Therapeutic strategies | |||
| Low-dose steroid monotherapy | 7 (6.5) | 0 | 0.361 |
| High-dose steroid monotherapy | 41 (38.3) | 5 (41.7) | 0.821 |
| Combination therapy, steroids + sDMARD | 29 (27.1) | 5 (41.7) | 0.290 |
| Combination therapy, steroids + biologics ± sDMARD | 30 (28.1) | 2 (16.6) | 0.400 |
↵* Statistical significance was expressed by p value < 0.005. Values in bold face indicate statistically significant results. MAS: macrophage activation syndrome; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; sDMARD: synthetic disease-modifying antirheumatic drugs; IQR: interquartile range.