Table 2.
Demographic and clinical features of the evaluated patients stratified according the different outcomes
| All patients | Monocyclic course | Polycyclic course | Chronic course | AOSD-related death | |
|---|---|---|---|---|---|
| Number of patients | 100 | 29 | 22 | 33 | 16 |
| Female/Male | 66/34 | 20/9 | 12/10 | 12/11 | 12/4 |
| Age (years), mean ± SD | 45.35 ± 16.23 | 49.31 ± 12.47 | 43.81 ± 15.13 | 40.24 ± 15.86 | 50.81 ± 19.97 |
| Clinical features | |||||
| Fever, n (%) | 100 (100) | 29 (100) | 22 (100) | 33 (100) | 16 (100) |
| Weight loss, n (%) | 5 (5) | 1 (3.44) | 2 (9.10) | 0 | 2 (12.5) |
| Rash, n (%) | 78 (78) | 24 (82.75) | 14 (48.27) | 28 (84.84) | 12 (75) |
| Joints involvement, n (%) | 86 (86) | 27 (93.10) | 21 (95.45) | 26 (78.79) | 12 (75) |
| Sore throat, n (%) | 64 (64) | 10 (34.48) | 13 (59.10) | 25 (75.76) | 16 (100) |
| Myalgia, n (%) | 57 (57) | 12 (41.37) | 15 (68.19) | 16 (48.49) | 14 (87.5) |
| Lymphadenopathy, n (%) | 57 (57) | 11 (37.93) | 16 (72.72) | 15 (45.45) | 15 (93.75) |
| Splenomegaly, n (%) | 79 (79) | 22 (75.86) | 20 (90.91) | 23 (69.70) | 14 (87.5) |
| Hepatic involvement, n (%) | 62 (62) | 18 (62.07) | 16 (72.72) | 18 (54.54) | 10 (62.5) |
| Pleurisy, n (%) | 14 (14) | 1 (3.44) | 5 (22.72) | 4 (12.12) | 4 (25) |
| Lung involvement, n (%) | 13(13) | 2 (6.89) | 3 (13.63) | 1 (3.03) | 7 (43.75) |
| Pericarditis, n (%) | 15 (15) | 1 (3.44) | 3 (13.63) | 6 (18.18) | 5 (31.25) |
| Abdominal pain, n (%) | 18 (18) | 2 (6.89) | 4 (18.19) | 7 (21.21) | 5 (31.25) |
| Systemic score, mean ± SD | 6.11 ± 2.02 | 4.93 ± 1.79 | 6.41 ± 2.05 | 6.12 ± 1.66 | 7.81 ± 1.54 |
| Comorbidities, n (%) | 33 (33) | 6 (20.68) | 8 (36.36) | 9 (27.28) | 10 (62.5) |
| Laboratory markers | |||||
| Leukocytosis > 15,000/mm3, n (%) | 36 (36) | 4 (13.79) | 7 (31.82) | 19 (57.57) | 6 (37.5) |
| Serum ferritin (ng/mL), mean ± SD | 2560.07 ± 3726.64 | 1634.82 ± 1198.92 | 2541.04 ± 3028.16 | 2886.09 ± 5438.65 | 3560.94 ± 3004.93 |
| ESR (mm/hour), mean ± SD | 67.28 ± 26.65 | 68.93 ± 23.94 | 61.81 ± 26.84 | 65.18 ± 26.77 | 74.93 ± 28.29 |
| CRP (mg/L), mean ± SD | 78.35 ± 72.76 | 83.83 ± 70.38 | 48.34 ± 35.64 | 72.16 ± 83.47 | 111.37 ± 61.76 |
| Complications | |||||
| MAS | 13 (13) | 0 | 3 (13.63) | 0 | 10 (62.5) |
| Kidney failure | 2 (2) | 0 | 0 | 0 | 2 (12.5) |
| Myocarditis | 1 (1) | 1 (3.44) | 0 | 0 | 0 |
| Treatments | |||||
| Low dosage of steroid monotherapy, n (%) | 4 (4) | 0 | 0 | 4 (12.12) | 0 |
| High dosage steroid monotherapy, n (%) | 39 (39) | 24 (79.31) | 2 (9.1) | 7 (21.21) | 6 (93.75) |
| High dosage steroid pulse therapy (500–1000 mg), n (%) | 37 (37) | 10 (34.48) | 9 (40.91) | 5 (15.15) | 13 (81.25) |
| sDMARD(s) | 55 (55) | 5 (17.24) | 18 (81.82) | 22 (66.67) | 10 (62.5) |
| Combination therapy, steroids + sDMARD(s), n (%) | 25 (25) | 5 (17.24) | 8 (36.36) | 10 (30.3) | 2 (12.5) |
| Combination therapy, steroids + biologics agent ± sDMARD(s), n (%) | 32 (32) | 0 | 12 (54.55) | 12 (36.37) | 8 (50) |
ESR erythrocyte sedimentation rate, CRP C-reactive protein, MAS macrophage activation, sDMARDs synthetic disease-modifying anti-rheumatic drugs