M694V^* (n = 89)

E148Q^** (n = 75)

V726A^*** (n = 68)

Other^**** (n = 89)

P-value

Age of onset (years), mean ± SD

3.7 ± 3.4

4.7 ± 3.4

5.2 ± 4

4.8 ± 3.6

0.068

Age at diagnosis (years), mean ± SD

6.5 ± 4.2

7.0 ± 3.8

7.8 ± 4.6

7.0 ± 4

0.30

Delay in diagnosis (years), mean ± SD

2.8 ± 2

2.3 ± 2

2.7 ± 2.5

2.5 ± 2

0.69

Abdominal pain

76 (85.4%)

64 (85.3%)

61 (89.7%)

71 (79.8%)

0.38

Fever

79 (88.8%)

51 (68%)

57 (83.8%)

64 (71.9%)

0.003

Chest pain

36 (40.4%)

13 (17.3%)

25 (36.8%)

24 (27%)

0.007

Arthralgia

46 (51.7%)

31 (41.3%)

25 (36.8%)

36 (40.4%)

0.24

>3 hospitalizations prior to diagnosis

4 (4.5%)

3 (4%)

4 (5.9%)

6 (6.7%)

0.78

Response to colchicine

0.08

No response

9 (10.1%)

11 (14.6%)

13 (19.1%)

23 (25.8%)

Incomplete response

47 (52.8%)

29 (38.7%)

29 (42.7%)

32 (36%)

Complete response

33 (37.1%)

35 (46.7%)

26 (38.2%)

34 (38.2%)

Severity score

0.005

Mild

25 (28.1%)

28 (37.3%)

32 (47.1%)

47 (52.8%)

Moderate and severe

64 (71.9%)

47 (62.7%)

36 (52.9%)

42 (47.2%)