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Table 1 Demographic characteristics and follow-up time of children with a clinical AID diagnosis without pathogenic gene variants

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

Female gender, N (%)

4 (44)

3 (38)

6 (43)

0 (0)

13 (39)

Mediterranean ethnicity, N (%)

8 (89)

5 (63)

7 (50)

1 (50)

21 (64)

Age at AID diagnosis in years, median (range)

3.6 (0.9–7.8)

3.8 (0.8–6.1)

3.3 (0.9–8.5)

9.1 (6.2–11.9)

3.6 (0.8–11.9)

Age at colchicine start in years, median (range)

3.6 (1.2–10)

3.9 (0.8–7.5)

3.5 (1.2–9.1)

9.5 (6.3–12.6)

3.8 (0.8–12.6)

Age at last follow-up in years, median (range)

5.6 (2.5–11.6)

5.0 (1.7–9.7)

5.0 (2.3–10.6)

10.5 (6.9–14.1)

5.3 (1.7–14.1)

Time baseline to last follow-up in months, median (range)

15.2 (8.7–34.2)

11.2 (5.3–47.3)

13.7 (7.8–47.3)

12.7 (6.9–18.4)

14.1 (5.3–47.3)

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