Table 2.
Comparison of clinical characteristics of SD patients with and without MAS.
| Characteristics | With MAS (n = 20) |
Without MAS (n = 186) |
p Value | |
|---|---|---|---|---|
| Epidemiology | ||||
| Age at diagnosis, median (IQR), y | 22.0 (12.0–27.0) | 29.0 (11.0–43.8) | 0.139 | |
| Sex (male), No. (%) | 7 (35.0%) | 79 (42.5%) | 0.685 | |
| Caucasian origin, No. (%) | 9/16 (56.2%) | 99/143 (69.2%) | 0.440 | |
| Immunosuppression, No. (%) | 7 (35.0%) | 5/174 (2.7%) | <0.001 | |
| Classification criteria | ||||
| ILAR, No. (%) | 3/8 (37.5%) | 24/55 (43.6%) | 1.000 | |
| PReS, No. (%) | 3/7 (42.9%) | 28/51 (54.9%) | 0.694 | |
| Fautrel, No. (%) | 9/10 (90.0%) | 74/118 (62.7%) | 0.098 | |
| Yamaguchi, No. (%) | 9/10 (90.0%) | 68/118 (57.6%) | 0.050 | |
| Virus tests | ||||
| Positive CMV PCR, No. (%) | 0/13 (0.0%) | 5/48 (10.4%) | 0.574 | |
| Positive EBV PCR, No. (%) | 4/15 (26.7%) | 10/51 (19.6%) | 0.720 | |
| Clinical features | ||||
| Fever, No. (%) | 19/19 (100.0%) | 165/178 (92.7%) | 0.619 | |
| Arthritis/arthralgia, No. (%) | 14/18 (77.8%) | 162/ 181 (89.5%) | 0.137 | |
| Skin rash, No. (%) | 14/19 (73.7%)) | 120/174 (69.0%) | 0.872 | |
| Splenomegaly, No. (%) | 2/19 (10.5%) | 19/140 (13.6%) | 1.000 | |
| Hepatomegaly, No. (%) | 6/19 (31.6%) | 15/136 (11.0%) | 0.025 | |
| Lymphadenopathy, No. (%) | 7/19 (36.8%) | 57/168 (33.9%) | 1.000 | |
| Digestive involvement, No. (%) | 4/19 (21.1%) | 30/169 (17.8%) | 0.754 | |
| Lung involvement, No. (%) | 3/19 (15.8%) | 32/171 (18.7%) | 1.000 | |
| Heart involvement, No. (%) | 6/19 (31.6%) | 32/170 (18.8%) | 0.225 | |
| Neurological involvement, No. (%) | 4 (20.0%) | 10 (5.4%) | 0.034 | |
| Treatments | ||||
| Steroids, No. (%) | 19 (95.0%) | 152/182 (83.5%) | 0.323 | |
| IL-1-blockers, No. (%) | 13 (65.0%) | 72/182 (39.6%) | 0.051 | |
| IL-6-blockers, No. (%) | 8 (40.0%) | 24/182 (13.2%) | 0.005 | |
| Immunosupressive drugs, No. (%) | 5 (25.0%) | 7/182 (3.85%) | 0.003 | |
| IVIg, No. (%) | 8 (40.0%) | 15/182 (8.2%) | <0.001 | |
| Evolution | ||||
| Recovery, No. (%) | 6 (30.0%) | 66/172 (38.4%) | 0.305 | |
| Relapse, No. (%) | 17 (85.0%) | 92/169 (54.4%) | 0.017 | |
| Death, No. (%) | 0 (0.0%) | 5/165 (3.0%) | 1.000 | |
| Disease course | ||||
| Monocyclic (systemic), No. (%) | 6 (30.0%) | 58/156 (37.2%) | 0.530 | |
| Polycyclic (systemic), No. (%) | 11 (55.0%) | 50/156 (32.1%) | 0.042 | |
| Chronic (articular), No. (%) | 3 (15.0%) | 48/156 (30.8%) | 0.193 | |
CMV, cytomegalovirus; EBV, Epstein–Barr virus; IL, interleukin; ILAR, International League of Associations for Rheumatology; IQR, interquartile range; IVIg, intravenous immunoglobulins; MAS, macrophage activation syndrome; PreS, Pediatric Rheumatology European Society; PCR, polymerase chain reaction.