FMF N = 9 | CAPS N = 8 | PFAPA N = 14 | unclassified AID N = 2 | Total cohort N = 33 | |
---|---|---|---|---|---|
Flare frequencies in categories: 0: no flare, 1: >every 6 weeks, 2: every 5–6 weeks, 3: every 3–4 weeks, 4: every 1–2 weeks, median (range) | |||||
Baseline | 2 (1–4) | 3 (1–4) | 3 (1–4) | 2 (1–3) | 3 (1–4) |
First follow-up | 1 (0–3) | 1 (0–3) | 0 (0–3) | 1 (0–1) | 1 (0–3) |
Last follow-up | 0 (0–3) | 1 (0–3) | 1 (0–3) | 1 (0–1) | 0 (0–3) |
Median flare duration in category: 0: no flare, 1: 1–2 days, 2: 3–4 days, 3: ≥5 days, median (range) | |||||
Baseline | 2 (1–3) | 3 (3) | 3 (2–3) | 3 (3) | 3 (1–3) |
First follow-up | 2 (0–3) | 2 (0–3) | 0 (0–3) | 2 (0–3) | 2 (0–3) |
Last follow-up | 0 (0–2) | 0 (0–3) | 2 (0–3) | 1 (0–1) | 0 (0–3) |
Patients with febrile flares, defined as ≥38.0° Celsius body temperature (orally, rectally, axillary, ear) during last flare, N (%) | |||||
Baseline, (tested) | 8 (89), (9/9) | 8 (100), (8/8) | 14 (100), (14/14) | 2 (100), (2/2) | 32 (97), (33/33) |
First follow-up, (tested) | 6 (67), (9/9) | 5 (63), (8/8) | 5 (42), (12/14) | 1 (50), (2/2) | 17 (55), (31/33) |
Last follow-up, (tested) | 2 (22), (9/9) | 4 (50), (8/8) | 6 (50), (12/14) | 1 (50), (2/2) | 13 (42), (31/33) |