M694V/M694V (n = 17)

M694V/Other (n = 72)

Other/Other (n = 232)

P-value

Age of onset (years), mean ± SD

3.9 ± 2.7

3.7 ± 3.5

4.9 ± 3.7

0.041

Age at diagnosis (years), mean ± SD

6.4 ± 3.7

6.618 ± 4.4

7.2 ± 4.2

0.426

Delay in diagnosis (years), mean ± SD

2.5 ± 2.3

2.9 ± 2

2.5 ± 2

0.592

Abdominal pain

15 (88.2%)

61 (84.7%)

196 (84.5%)

0.917

Fever

15 (88.2%)

64 (88.9%)

172 (74.1%)

0.018

Chest pain

7 (41.2%)

29 (40.3%)

62 (26.7%)

0.057

Arthralgia

12 (70.6%)

34 (47.2%)

92 (39.7%)

0.032

>3 hospitalizations prior to diagnosis

3 (17.6%)

1 (1.4%)

13 (5.6%)

0.015

Response to colchicine

0.069

No response

2 (11.8%)

7 (9.7%)

47 (20.3%)

Incomplete response

11 (64.7%)

36 (50%)

90 (38.8%)

Complete response

4 (23.5%)

9 (40.3%)

95 (40.9%)

Severity score

0.008

Mild

3 (17.6%)

22 (30.5%)

107 (46.1%)

Moderate and severe

14 (82.4%)

50 (69.5%)

125 (53.9%)