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Table 46 (abstract P165). See text for description

From: Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part one

Patient

1

2

3

4

5

6

Age (years), gender

11, m

2, m

1, f

8, f

15, f

11, m

Systemic features

Spiking fever, rash,

Spiking fever, rash, hepatomegaly, splenomegaly

Spiking fever, rash, hepatomegaly, splenomegaly

Spiking fever, rash

Spiking fever, rash

Spiking fever, rash, hepatomegaly, splenomegaly

Joint involvement

Arthralgia

Arthralgia

Oligoarthritis

Poly-arthritis

Oligo-arthritis

Arthralgia

ESR, mm/h

45

68

na

na

63

na

CRP, mg/l

95.6

101.7

106.6

197.0

329.8

99.0

IL18, pg/ml

2,483

>10,000

>5,000

>10,000

>10,000

>5,000

Pretreatment

3 days corticosteroids

none

none

none

none

3 days corticosteroids

Initial response

Immediate complete response

Immediate complete response

Immediate partial response

none

Improvement of arthritis, suppression of fever

Immediate complete response

Outcome

Remission off treatment

Remission off treatment

Increase of dosage upon flare, discontinued with MAS

No response, switch to Tocilizumab

Minor response, switch to Tocilizumab

Remission, relapse after discontinuation