Table 2.
Frequency of clinical features related to Still’s disease among patients with pediatric, adult and elderly onset
| Pediatric onset (n=65) |
Adult onset (n=314) |
Elderly onset (n=32) |
P value | |
| Sore throat, n (%) | 31 (47.7) | 170 (54.1) | 16 (50) | 0.60 |
| Salmon coloured skin rash, n (%) | 50 (76.9) | 179 (57) | 18 (56.3) | 0.01* |
| Atypical skin rash, n (%) | 13 (20) | 79 (25.2) | 3 (9.4) | 0.11 |
| Splenomegaly, n (%) | 21 (32.3) | 103 (32.8) | 9 (28.1) | 0.86 |
| Liver involvement, n (%) | 18 (27.7) | 105 (33.4) | 13 (40.6) | 0.43 |
| Lymphadenopathy, n (%) | 36 (55.3) | 144 (45.6) | 17 (32.1) | 0.31 |
| Thoracic pain, n (%) | 2 (3.1) | 33 (10.5) | 3 (9.4) | 0.17 |
| Pneumonia, n (%) | 3 (4.6) | 17 (5.4) | 2 (6.3) | 0.94 |
| Pleuritis, n (%) | 2 (3.1) | 46 (14.6) | 6 (18.8) | 0.02† |
| Pericarditis, n (%) | 8 (12.3) | 36 (11.5) | 6 (18.8) | 0.49 |
| Abdominal pain, n (%) | 16 (24.6) | 35 (11.1) | 3 (9.4) | 0.01* |
| Peritonitis, n (%) | 1 (1.5) | 2 (0.6) | 0 (0.0) | 0.65 |
| Myalgia, n (%) | 30 (46.2) | 151 (48.1) | 16 (50) | 0.93 |
| Arthralgia, n (%) | 58 (89.2) | 254 (80.9) | 21 (65.6) | 0.02† |
| Arthritis, n (%) | 44 (67.7) | 154 (49) | 18 (56.3) | 0.02* |
| Monarthritis, n (%) | 5 (11.3) | 6 (5.9) | 1 (5.6) | 0.16 |
| Oligoarthritis, n (%) | 17 (38.6) | 61 (39.6) | 9 (50) | 0.67 |
| Polyarthritis, n (%) | 17 (38.6) | 75 (48.6) | 6 (33.3) | 0.28 |
| MAS,‡ n (%) | 11 (16.9) | 25 (7.9) | 1 (3.1) | 0.03 |
| ARDS, n (%) | 0 (0) | 5 (1.6) | 1 (3.1) | 0.44 |
| Fulminant hepatitis, n (%) | 0 (0) | 2 (0.6) | 0 (0) | 0.73 |
| Cardiac complications, n (%) | 3 (4.6) | 5 (1.6) | 0 (0) | 0.19 |
| Vascular complications, n (%) | 0 (0) | 4 (1.3) | 0 (0) | 0.54 |
| New disease manifestations developed over time, n (%) | 6 (9.2) | 37 (11.8) | 2 (6.3) | 0.56 |
P values were obtained with the χ2 test; post-hoc analysis was performed with the χ2 test or with the Fisher exact test according to the expected frequencies.
*Refers to a statistically significant differences after Bonferroni correction (p<0.017) between the pediatric-onset group and the adult-onset group.
†Refers to a statistically significant differences at post-hoc analysis after Bonferroni correction (p<0.017) between the pediatric-onset group and the elderly-onset group.
‡MAS was diagnosed in all cases with the criteria proposed by Ravelli et al.20 Three patients fulfilled the HLH-2004 criteria.22
ARDS, acute respiratory distress syndrome; MAS, macrophage activation syndrome; n, number.