Table 1

Demographic data, duration of treatment, schedule (dose and frequency) of canakinumab and response to treatment at the beginning of the follow-up period

Patient number

Phenotype

NLRP3

mutation

Disease

onset

Duration of treatment (months)

Body weight

(Kg)

Dose

(mg/kg)

Dose at each

Administration, mg

Canakinumab

frequency, weeks

Response


1

CINCA

N477K

birth

5

65

4.6

300

8

partial

2

CINCA

F573S

birth

5

38

4.0

152

8

inadequate

3

CINCA

Negative

2 months

5

50

6.0

300

8

partial

4

CINCA

Negative

birth

6

54

2.8

150

8

partial

5

MWS/CINCA

D303N/V198M

1 week

12

29

2.0

72

8

complete

6

MWS/CINCA

T348M

6 months

11

85

3.5

300

8

partial

7

CINCA

E304K

birth

12

78

3.8

300

8

partial

8

MWS

T348M

birth

12

63

2.4

150

8

partial

9

MWS

E525K

15 months

17

62

2.4

150

8

complete

10

MWS

D303N

3 weeks

20

37

2.0

74

8

complete

11

CINCA

T348M

birth

15

23

2.0

46

8

partial

12

CINCA

Negative

birth

7

61

2.5

150

8

complete

13

MWS

V198M

9 years

-

38

2.0

76

8

complete


CINCA: chronic infantile neurological cutaneous articular syndrome; MWS: Muckle-Wells syndrome; MWS/CINCA: overlap syndrome.

Caorsi et al. Arthritis Research & Therapy 2013 15:R33   doi:10.1186/ar4184

Open Data