Table 2 Evaluation of disease activity score or clinical symptoms in 16 patients

1

AGS^a

3

3

3

3

3

3

2

AGS^a

1.26

3

3

3

3

3

3

AGS^a

0.2

0.2

0

0

-

-

4

Blau syndrome^b

0

0

0

0

0

0

5

Blau syndrome^b

6

12

12

-

-

-

6

Blau syndrome^b

0

0

0

0

0

0

7

Blau syndrome^b

30

30

12

4

-

-

8

CINCA^c

35

28

10

8

2

4

9

CINCA^c

7

7

14

5

0

0

10

DADA2

Persistent fever, rash, oral ulcers and celialgia

Persistent fever, rash, oral ulcers and celialgia

Reduced fever frequency

Fever and celialgia improved

Few ulcers and rashes remained

Disappearance of rash

11

FMF^c

15

15

0

0

0

0

12

HA20^d

Mild

Mild

Disease-free activity

Disease-free activity

Disease-free activity

Disease-free activity

13

HA20^d

Moderate

Moderate

Mild

Disease-free activity

Moderate

Disease-free activity

14

PLAID

Recurrent fevers and rash

Recurrent fevers and rash

Intermittent fever, rash and arthralgia

Intermittent fever, rash and arthralgia

Occasional fever and rash

Occasional fever and rash

15

SAVI^e

3

3

1

2

2

-

16

TRAPS^c

15

15

15

10

15

10