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
| Patient | 1 | 2 | 3 |
|---|---|---|---|
| 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 |