Demographic characteristics of adult-onset Still's disease and systemic juvenile idiopathic arthritis patients included in the study
| (%) . | AOSD act . | AOSD rem . | sJIA act . | sJIA rem . | MAS . |
|---|---|---|---|---|---|
| n | 23 | 25 | 3 | 6 | 8* |
| Age | 48.7 (19.5) | 45.8 (20.2) | 6.0 (3.6) | 8.7 (3.9) | 46.1 (20) |
| Female n | 14 (60.1) | 15 (60.0) | 2 (66.7) | 5 (83.3) | 8 (100) |
| Fulfilling MAS criteria | 4 (17.3) | 0 0 | 0 0 | 0 0 | 8 (100) |
| Pouchot score | 5.1 (2.9) | 0.0 (0.0) | 2.0 (1.0) | 0.0 (0.0) | |
| High-grade fever | 16 (70.0) | 0 0 | 0 0 | 0 0 | 8 (100) |
| Evanescent rash | 16 (70.0) | 0 0 | 2 (66.7) | 0 0 | 0 0 |
| Arthralgia | 15 (65.2) | 0 0 | 0 0 | 0 0 | 0 0 |
| Leucocytosis | 7 (30.4) | 0 0 | 1 (33.3) | 0 0 | 0 0 |
| Sore throat | 9 (39.1) | 0 0 | 0 0 | 0 0 | 0 0 |
| Lymphadenopathy or splenomegaly | 10 (43.5) | 0 0 | 0 0 | 0 0 | 5 (62.5) |
| Liver dysfunction | 19 (82.6) | 0 0 | 0 0 | 0 0 | 7 (87.5) |
| RF/ANA negative | 22 (95.7) | 19 (76) | 3 (100) | 6 (100) | 3 (37.5) |
| Hyperferritinemia >1000 ng/ml | 15 (65.2) | 0 0 | 0 0 | 0 0 | 8 (100) |
| (%) . | AOSD act . | AOSD rem . | sJIA act . | sJIA rem . | MAS . |
|---|---|---|---|---|---|
| n | 23 | 25 | 3 | 6 | 8* |
| Age | 48.7 (19.5) | 45.8 (20.2) | 6.0 (3.6) | 8.7 (3.9) | 46.1 (20) |
| Female n | 14 (60.1) | 15 (60.0) | 2 (66.7) | 5 (83.3) | 8 (100) |
| Fulfilling MAS criteria | 4 (17.3) | 0 0 | 0 0 | 0 0 | 8 (100) |
| Pouchot score | 5.1 (2.9) | 0.0 (0.0) | 2.0 (1.0) | 0.0 (0.0) | |
| High-grade fever | 16 (70.0) | 0 0 | 0 0 | 0 0 | 8 (100) |
| Evanescent rash | 16 (70.0) | 0 0 | 2 (66.7) | 0 0 | 0 0 |
| Arthralgia | 15 (65.2) | 0 0 | 0 0 | 0 0 | 0 0 |
| Leucocytosis | 7 (30.4) | 0 0 | 1 (33.3) | 0 0 | 0 0 |
| Sore throat | 9 (39.1) | 0 0 | 0 0 | 0 0 | 0 0 |
| Lymphadenopathy or splenomegaly | 10 (43.5) | 0 0 | 0 0 | 0 0 | 5 (62.5) |
| Liver dysfunction | 19 (82.6) | 0 0 | 0 0 | 0 0 | 7 (87.5) |
| RF/ANA negative | 22 (95.7) | 19 (76) | 3 (100) | 6 (100) | 3 (37.5) |
| Hyperferritinemia >1000 ng/ml | 15 (65.2) | 0 0 | 0 0 | 0 0 | 8 (100) |
Act: active; ANA: antinuculear antibody; AOSD: adult-onset Still's disease; MAS: macrophage activation syndrome; rem: remission. *includes patients with systemic lupus erythematosus (SLE; n = 4), virus associated hemophagocytic syndrome (HPS; n = 3) and lymphoma associated HPS (n = 1).
Demographic characteristics of adult-onset Still's disease and systemic juvenile idiopathic arthritis patients included in the study
| (%) . | AOSD act . | AOSD rem . | sJIA act . | sJIA rem . | MAS . |
|---|---|---|---|---|---|
| n | 23 | 25 | 3 | 6 | 8* |
| Age | 48.7 (19.5) | 45.8 (20.2) | 6.0 (3.6) | 8.7 (3.9) | 46.1 (20) |
| Female n | 14 (60.1) | 15 (60.0) | 2 (66.7) | 5 (83.3) | 8 (100) |
| Fulfilling MAS criteria | 4 (17.3) | 0 0 | 0 0 | 0 0 | 8 (100) |
| Pouchot score | 5.1 (2.9) | 0.0 (0.0) | 2.0 (1.0) | 0.0 (0.0) | |
| High-grade fever | 16 (70.0) | 0 0 | 0 0 | 0 0 | 8 (100) |
| Evanescent rash | 16 (70.0) | 0 0 | 2 (66.7) | 0 0 | 0 0 |
| Arthralgia | 15 (65.2) | 0 0 | 0 0 | 0 0 | 0 0 |
| Leucocytosis | 7 (30.4) | 0 0 | 1 (33.3) | 0 0 | 0 0 |
| Sore throat | 9 (39.1) | 0 0 | 0 0 | 0 0 | 0 0 |
| Lymphadenopathy or splenomegaly | 10 (43.5) | 0 0 | 0 0 | 0 0 | 5 (62.5) |
| Liver dysfunction | 19 (82.6) | 0 0 | 0 0 | 0 0 | 7 (87.5) |
| RF/ANA negative | 22 (95.7) | 19 (76) | 3 (100) | 6 (100) | 3 (37.5) |
| Hyperferritinemia >1000 ng/ml | 15 (65.2) | 0 0 | 0 0 | 0 0 | 8 (100) |
| (%) . | AOSD act . | AOSD rem . | sJIA act . | sJIA rem . | MAS . |
|---|---|---|---|---|---|
| n | 23 | 25 | 3 | 6 | 8* |
| Age | 48.7 (19.5) | 45.8 (20.2) | 6.0 (3.6) | 8.7 (3.9) | 46.1 (20) |
| Female n | 14 (60.1) | 15 (60.0) | 2 (66.7) | 5 (83.3) | 8 (100) |
| Fulfilling MAS criteria | 4 (17.3) | 0 0 | 0 0 | 0 0 | 8 (100) |
| Pouchot score | 5.1 (2.9) | 0.0 (0.0) | 2.0 (1.0) | 0.0 (0.0) | |
| High-grade fever | 16 (70.0) | 0 0 | 0 0 | 0 0 | 8 (100) |
| Evanescent rash | 16 (70.0) | 0 0 | 2 (66.7) | 0 0 | 0 0 |
| Arthralgia | 15 (65.2) | 0 0 | 0 0 | 0 0 | 0 0 |
| Leucocytosis | 7 (30.4) | 0 0 | 1 (33.3) | 0 0 | 0 0 |
| Sore throat | 9 (39.1) | 0 0 | 0 0 | 0 0 | 0 0 |
| Lymphadenopathy or splenomegaly | 10 (43.5) | 0 0 | 0 0 | 0 0 | 5 (62.5) |
| Liver dysfunction | 19 (82.6) | 0 0 | 0 0 | 0 0 | 7 (87.5) |
| RF/ANA negative | 22 (95.7) | 19 (76) | 3 (100) | 6 (100) | 3 (37.5) |
| Hyperferritinemia >1000 ng/ml | 15 (65.2) | 0 0 | 0 0 | 0 0 | 8 (100) |
Act: active; ANA: antinuculear antibody; AOSD: adult-onset Still's disease; MAS: macrophage activation syndrome; rem: remission. *includes patients with systemic lupus erythematosus (SLE; n = 4), virus associated hemophagocytic syndrome (HPS; n = 3) and lymphoma associated HPS (n = 1).
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