Table 1

Demographic, clinical and histopathologic features of patients with MAS and with active sJIA without MAS*

FeatureMAS
(n=362)
sJIA without MAS
(n=404)
P value
Females208 (57.5)203 (50.2)0.13
Median (IQR) age at onset of sJIA, years5.3 (2.7–10.1)5.5 (2.5–9.5)0.80
Fever341/355 (96.1)382/403 (94.8)0.79
Evanescent skin rash217/348 (62.4)299/402 (74.4)0.0004
Hepatomegaly245/350 (70)123/400 (30.8)<0.0001
Splenomegaly201/347 (57.9)95/399 (23.8)<0.0001
Lymphadenopathy178/346 (51.4)115/396 (29)<0.0001
Active arthritis230/354 (65)382/401 (95.3)<0.0001
Central nervous system disease122/349 (35)7/400 (1.8)<0.0001
Haemorrhagic manifestations71/348 (20.4)5/402 (1.2)<0.0001
Bone marrow hemophagocytosis149/249 (59.8)
  • Davì et al, Arthritis Rheumatol 2014;66:2871–80.

  • *Data are number positive/number with information available (%) unless otherwise indicated.

  • MAS, macrophage activation syndrome; sJIA, systemic juvenile idiopathic arthritis.