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 |