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Table 6 Flare response of children with a clinical AID diagnosis without pathogenic gene variants and colchicine

From: Colchicine – an effective treatment for children with a clinical diagnosis of autoinflammatory diseases without pathogenic gene variants

 

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)

  1. Abbreviations: AID autoinflammatory disease, FMF Familial Mediterranean Fever, CAPS Cryopyrin-associated periodic syndromes, PFAPA periodic fever, aphthosis, pharyngitis and adenitis