Table 1

Features of patients with AOSD with and without MAS*

non-MAS (n=174)MAS (n=35)P values
Demographic
 Age, mean±SD (years)36.9±13.532±11.40.028
 Gender (F/M)138/3628/70.927
 Deaths, n (%)6 (3.4)6 (17.1)0.001
Clinical manifestations
 Arthritis, n (%)119 (68.4)25 (71.4)0.723
 Eruption, n (%)123 (70.7)28 (80)0.262
 Abnormal liver function, n (%)143 (82.2)33 (94.3)0.073
 Decreased blood cells, n (%)1 (0.6)26 (74.3)<0.001
 Central nervous system involvement, n (%)0 (0)7 (20)<0.001
 Haemorrhagic manifestations, n (%)0 (0)6 (17.1)<0.001
 Splenomegaly, n (%)36 (20.7)8 (22.9)0.774
 Hepatomegaly, n (%)3 (1.7)4 (11.4)0.016
 Enlarged lymph nodes, n (%)107 (61.5)28 (80)0.037
 Known underlying immunosuppression1 (0.6)13 (37.1)<0.001
 Temperature (°C)
  38.4–39.451 (29.3)3 (8.6)0.011
  >39.4123 (70.7)32 (91.4)0.011
 Bone marrow Hemophagocytosis1 (0.6)17 (48.6)<0.001
Laboratory features
 White cell count (×109/L)14.2 (3.6–50.4)6.3 (0.2–37.7)<0.001
 Neutrophil count (×109/L)12.1 (1.13–48.13)5.2 (0–36.3)<0.001
 Lymphocyte count (×109/L)1.28 (0.4–4.71)0.7 (0.15–3.08)<0.001
 Haemoglobin (g/L)109 (53–141)85 (63–134)<0.001
 Platelet count (×109/L)295 (34–564)81 (8–368)<0.001
 Ferritin (ng/mL)1813 (25–42 138)2000 (459–217 988)0.007
 Aspartate aminotransferase (U/L)40 (7–555)157 (17–2888)<0.001
 Alanine aminotransferase (U/L)37 (5–539)143 (11–2407)<0.001
 Triglycerides (mmol/L)1.2 (0.4–3.8)2.56 (0.7–19.3)<0.001
 Fibrinogen (g/L)4.3 (0.9–8.1)1.49 (0.31–5.59)<0.001
 ESR (mm/hour)69 (3–132)27 (1–126)<0.001
 CRP (mg/L)83.0 (0.27–498.9)75.6 (1.5–250)0.772
Scores
 HScore, median (range)68 (33-156)196 (98–333)<0.001
 MS score, median (range)−1.17 (−1.26 to 2.52)1.05 (−1.26 to 26.55)<0.001
  • *Values are expressed as n (%) or median (range).

  • AOSD, adult-onset Still’s disease; CRP, C reactive protein; ESR, erythrocyte sedimentation rate; F, female; M, male; MAS, macrophage activation syndrome.