Baseline demographic and disease characteristics (safety set)*
| Characteristics | Patients (n=60) |
| Median age, years (Q1, Q3) | 18.0 (14.0 to 29.5) |
| Female, n (%) | 28 (46.7) |
| Caucasian, n (%) | 49 (81.7) |
| Median duration of disease, years (Q1, Q3) | 13.8 (9.3 to 24.2) |
| Median number of flares per year (Q1, Q3) | 17.5 (12.0 to 27.5) |
| Active disease at maximum colchicine dose, n (%) | 59 (98.3) |
| CRP (mg/L), median (Q1, Q3) | 102 (56.7 to 202.6) |
| SAA (mg/L), median (Q1, Q3) | 618 (265.5 to 1266.0) |
| PGA score (disease activity), n (%) | |
| 0 (None) | 0 |
| 1 (Minimal) | 0 |
| 2 (Mild) | 9 (15.0) |
| 3 (Moderate) | 34 (56.7) |
| 4 (Severe) | 17 (28.3) |
| Arthralgia/arthritis, n (%) | |
| None | 21 (35.0) |
| Minimal | 8 (13.3) |
| Mild | 12 (20.0) |
| Moderate | 16 (26.7) |
| Severe | 3 (5.0) |
| MEFV genotype, n (%) | |
| M694V/M694V | 42 (70.0) |
| M694V/M694I | 3 (5.0) |
| Other genotypes with mutations in exon 10 | 13 (21.7) |
| No mutations in exon 10† | 2 (3.3) |
| Prior use of biologics, n (%) | 16 (26.7) |
| Anakinra | 15 (25.0) |
*Baseline characteristics at study entry (day 0) for crFMF patients of the safety set of Epoch 4 (from week 41 to week 113). As described in the methods, active disease was an eligibility criterion to enter the study.
†Two Japanese patients with no mutations in exon 10 were included in the CLUSTER trial, but not randomised in Epoch 2 and treated with open-label canakinumab. These patients were included in all analyses of the safety population in Epoch 4.
crFMF, colchicine-resistant familial Mediterranean fever; CRP, C-reactive protein; n, number of patients; PGA, physician global assessment; SAA, serum amyloid A.