Table 5 Colchicine outcome in children with a clinical AID diagnosis without pathogenic gene variants
FMF N = 9 | CAPS N = 8 | PFAPA N = 14 | unclassified AID N = 2 | |
|---|---|---|---|---|
Complete response defined as PGA ≤2 cm plus CRP ≤0.5 mg/dL and/or SAA ≤10 mg/L, % (confidence interval) | ||||
First follow-up, N (tested) | 43 (14.3; 76.4), 3 (7/9) | 43 (14.3; 82.4), 3 (7/8) | 75 (58.3; 100.0), 9 (12/14) | 100 (100.0; 100.0), 2 (2/2) |
Last follow-up, N (tested) | 63 (37.5; 95.9), 5 (8/9) | 50 (25.0; 89.2), 4 (8/8) | 54 (30.8; 81.4), 7 (13/14) | 50 (50.0; 100.0), 1 (2/2) |
Partial response defined as PGA > 2 ≤ 5 cm plus CRP > 0.5 mg/dL ≤5 mg/dL and/or SAA > 10 mg/L ≤ 50 mg/L, % (confidence interval) | ||||
First follow-up, N (tested) | 57 (28.6; 90.7), 4 (7/9) | 43 (14.3; 82.4), 3 (7/8) | 17 (0.0; 42.0), 2 (12/14) | 0 (0.0; 85.6), 0 (2/2) |
Last follow-up, N (tested) | 37 (12.5; 70.9), 3 (8/9) | 38 (12.5; 76.7), 3 (8/8) | 31 (7.7; 58.3), 4 (13/14) | 50 (50.0; 100.0), 1 (2/2) |
No response defined as PGA > 5 and/or CRP > 5 mg/dL and/or SAA > 50 mg/L, % (confidence interval) | ||||
First follow-up, N (tested) | 0, 0 (7/9) | 14 (0.0; 53.8), 1 (7/8) | 8 (0.0; 33.6), 1 (12/14) | 0, 0 (2/2) |
Last follow-up, N (tested) | 0, 0 (8/9) | 12 (0.0; 51.7), 1 (8/8) | 15 (0.0; 42.9), 2 (13/14) | 0, 0 (2/2) |
Requirement of anti-IL-1 treatment, N (%) | ||||
First follow-up | 1 (7) | |||
Last follow-up | 1 (7) | |||
Colchicine impact of flares, N (%) reduced flare frequency or shorter flares or lower/no fever | ||||
First follow-up, (tested) | 7 (78), (9/9) | 5 (71), (7/8) | 12 (92), (13/14) | 1 (50), (2/2) |
Last follow-up, (tested) | 9 (100), (9/9) | 7 (100), (7/8) | 10 (83), (12/14) | 2 (100), (2/2) |