Table 1.
Reports of Canakinumab use in AOSD patients.
| Reports | # Patients and dosage | Results |
|---|---|---|
| Kontzias and Efthimiou, 2012 | n. 2 patients with refractory AOSD received 150 mg of Canakinumab s.c. injections every 8 weeks | – Resolved systemic symptoms, – normalized laboratory values, – no AEs (except transient diarrhea in 1 of 2 patients), – no relapse in 6–12 months |
| Banse et al., 2013 | n. 1 patient with refractory AOSD received two doses of 150 mg of Canakinumab s.c. injections per week | – Development of MAS after the second injection at 3 and 6 months after MAS, – resolved symptoms and normalized laboratory values |
| Eriksson et al., 2013 | n. 1 patient with refractory AOSD received 150 mg of Canakinumab s.c. injections every 8 weeks | – Resolved joint and skin symptoms – normalized laboratory values |
| Barsotti et al., 2014 | n. 1 patient with refractory AOSD received 150 mg of Canakinumab s.c. injections every 4 weeks, gradually increasing up to 150 mg every 8 weeks | – Three brief episodes of fever, not temporally related to Canakinumab – no systemic and joint symptoms at 18 month follow-up |
| Lo Gullo et al., 2014 | n. 1 patient with refractory AOSD received 150 mg of Canakinumab s.c. injections every 8 weeks | – No systemic symptoms at 14 month follow-up – normalized laboratory values – recurrent arthritis required methylprednisolone 12 mg/day |
| Maria et al., 2014 | n. 1 patient with refractory AOSD received Canakinumab (dosage not reported) | – Rapid and complete remission of systemic symptoms at 30 month follow-up |
| Rossi-Semerano et al., 2015 | A French nationwide survey includes n. 2 patients with refractory AOSD receiving 150 mg of Canakinumab s.c. injections every 4 and 8 weeks, respectively | – Complete remission in 1 patient receiving 150 mg/8 week – no clinical improvement in 1 patient receiving 150 mg/4 week |
| Colafrancesco et al., 2017 | An Italian nationwide survey includes n. 4 patients with refractory AOSD receiving 150 mg of Canakinumab s.c. injections every 8 weeks | – Normalized laboratory values – effective in 3 out 4 patients – loss of efficacy in 1 patient with chronic articular AOSD – no AEs |
| Feist et al., 2018 | Pooled analyses from SoJIA studies (NCT00426218, NCT00886769, NCT00889863, and NCT00891046) include n. 29 older adolescents/young adults (representing AOSD patients), receiving 4 mg/kg every 4 weeks | – At day 15, 19 out 29 older adolescents/young adults have aACR ≥ 70 responses and showed improvements (13 out 18) at day 85. – Normalized laboratory values |
| Sinha et al., 2018 | n. 1 patient with refractory AOSD and severe pulmonary hypertension received Canakinumab (dosage not reported) | – No clinical improvement |
| Trial NCT02204293 (Fase II) | A 12-week trial including 68 AOSD patients randomized into two groups receiving either placebo or 150 mg of Canakinumab s.c. injections every 8 weeks. The aim of the study is to assess the efficacy, safety, and tolerability of Canakinumab | – The primary outcome measures will be available in June 2019 |
s.c., sub-cutaneous; AOSD, Adult-onset Still’s disease; AEs, adverse events; MAS, macrophage activation syndrome; SoJIA, systemic onset juvenile idiopathic arthritis; aACR, adapted American College of Rheumatology.