Table 1.
Clinical variables of assessed patients with Still’s disease
| Clinical characteristics | Cluster 1 ‘Juvenile/transitional’ |
Cluster 2 ‘Uncomplicated’ | Cluster 3 ’Hyperferritinemic’ |
Cluster 4 ‘Catastrophic’ |
Coefficient, p value |
| Number of patients | 115 | 128 | 31 | 75 | |
| Clinical variables for clusters analysis | |||||
| Age, years, mean±SD | 25.4±12.7 | 39.8±15.2 | 36.9±16.9 | 51.8±16.1 | 0.30, <0.0001 |
| Systemic score, mean±SD | 6.1±1.7 | 4.6±1.4 | 5.5±1.9 | 6.8±2.0 | 0.21, <0.0001 |
| ESR, mm/hour, mean±SD | 96.9±23.4 | 45.5±21.7 | 70.2±30.3 | 93.6±23.6 | 0.50, <0.0001 |
| CRP, mg/L, mean±SD | 36.1±37.8 | 46.3±43.5 | 146.6±101.1 | 143.8±103.6 | 0.35, <0.0001 |
| Ferritin, ng/mL, mean±SD | 2171.4±2279.6 | 1581.3±2253.9 | 17245.4±5328.1 | 2947.7±2818.6 | 0.71, <0.0001 |
| Other clinical features | |||||
| Male sex, n (%) | 46 (40.0) | 63 (49.2) | 10 (32.3) | 41 (54.7) | 6.83, 0.078 |
| Fever, n (%) | 111 (96.5) | 124 (96.9) | 30 (96.8) | 74 (98.7) | 1.73, 0.631 |
| Joint involvement, n (%) | 103 (89.6) | 117 (91.4) | 27 (87.1) | 66 (88.0) | 0.87, 0.834 |
| Arthralgia, n (%) | 101 (87.8) | 113 (88.3) | 26 (83.9) | 60 (80.0) | 3.22, 0.359 |
| Arthritis, n (%) | 71 (61.7) | 83 (64.8) | 17 (54.8) | 46 (61.3) | 1.13, 0.770 |
| Skin rash, n (%) | 83 (72.2) | 77 (60.2) | 21 (67.7) | 55 (73.3) | 5.36, 0.147 |
| Sore throat, n (%) | 70 (60.9) | 61 (47.7) | 18 (58.1) | 54 (72.0) | 12.25, 0.007 |
| Myalgia, n (%) | 75 (65.2) | 65 (50.8) | 17 (54.8) | 52 (69.3) | 9.19, 0.027 |
| Lymph node involvement, n (%) | 62 (53.9) | 51 (39.8) | 14 (45.2) | 48 (64.0) | 11.99, 0.007 |
| Liver involvement, n (%) | 58 (50.4) | 52 (40.6) | 18 (58.1) | 53 (70.7) | 18.20, <0.0001 |
| Spleen involvement, n (%) | 57 (49.6) | 44 (34.4) | 11 (35.5) | 45 (60.0) | 13.73, 0.003 |
| Pericarditis, n (%) | 18 (15.7) | 15 (11.7) | 7 (22.6) | 23 (30.7) | 12.57, 0.006 |
| Pleuritis, n (%) | 16 (13.9) | 14 (10.9) | 6 (19.4) | 26 (34.7) | 19.61, <0.0001 |
| Abdominal pain, n (%) | 21 (18.3) | 14 (10.9) | 3 (9.7) | 12 (16.0) | 3.14, 0.371 |
| Life-threatening complications | |||||
| MAS, n (%) | 21 (18.3) | 9 (7.0) | 7 (22.6) | 17 (22.7) | 11.82, 0.008 |
| Lung disease, n (%) | 9 (7.8) | 5 (3.9) | 2 (6.5) | 16 (21.3) | 17.98, <0.0001 |
| Therapies | |||||
| GCs, n (%) | 109 (94.8) | 122 (95.3) | 29 (93.5) | 71 (94.7) | 1.29, 0.730 |
| Low dose GCs, n (%) | 46 (42.2) | 64 (52.5) | 11 (37.9) | 28 (39.4) | 5.23, 0.156 |
| csDMARDs, n (%) | 68 (59.1) | 85 (66.4) | 22 (71.0) | 51 (68.0) | 1.79, 0.617 |
| bDMARDs, n (%) | 62 (53.9) | 57 (44.5) | 13 (41.9) | 30 (40.0) | 6.76, 0.344 |
| Disease courses and time of observation | |||||
| Monocyclic, n (%) | 49 (42.6) | 43 (33.6) | 18 (58.1) | 20 (26.7) | 20.55, 0.015 |
| Polycyclic, n (%) | 37 (32.2) | 46 (35.9) | 9 (29.0) | 30 (40.0) | |
| Chronic, n (%) | 27 (23.5) | 31 (24.2) | 4 (12.9) | 16 (21.3) | |
| Mortality, n (%) | 2 (1.7) | 8 (6.25) | 0 (0.0) | 9 (12.0) | |
| Follow-up, years, mean±SD | 2.57±3.55 | 3.35±3.42 | 2.35±2.77 | 3.44±3.33 | 0.01, 0.18 |
Bold values are statistically significant results.
bDMARDs, biological disease-modifying antirheumatic drugs; CRP, C reactive protein; csDMARDs, conventional synthetic disease-modifying antirheumatic drugs; ESR, erythrocyte sedimentation rate; GCs, glucocorticoids; MAS, macrophage activation syndrome.