Table 1 Demographics of the patients, clinical and laboratory data at time of diagnosis of sJIA, and time and dose of anti-IL-1 medication

Gender

m

m

f

ANA/RF/HLA-B27

neg/neg/neg

neg/neg/n.d.

neg/neg/neg

Age at diagnosis

14.8

15.8

6.8

ILAR criteria for subtype

Fever, typical rash, splenomegaly, pericardial effusion

Fever, rash, pericardial/pleural effusion, hepatomegaly

Fever, rash, pleural effusion, hepatomegaly

splenomegaly

Affected joints

Polyarthritis

Polyarthritis

Elbows, hips, wrists

Leukocyte count

9100/mm³

(4800–10000)

9100/mm³

(4500–12500)

16800/mm³

(4800–10000)

Platelet count

294000/mm³

(150000–450000)

208000/mm³

(154000–386000)

575000/mm³

(150000–450000)

Ferritin

2926 μg/l

(30–400)

32722 ng/ml

(22–322)

297–1197 μg/l

(30–400)

C-reactive protein

7.4 mg/dl

(0.2–1.0)

28.54 mg/dl

(<0.3)

3.36 mg/dl

(0.2–1.0)

Bone marrow biopsy findings

Negative

MAS

-

Start of IL1-treatment, years after diagnosis

0 years

2.2 years

0.3 years

IL-1 agents, initial dose

Anakinra

1.3 mg/kg

Canakinumab

1.8 mg/kg

Anakinra

1.1 mg/kg