Table 2.
Genotype-phenotype correlation
| High penetrance MEFV mutations | 2 | 1 | 0 |
|---|---|---|---|
| Number of patients (%) | n=21 (68%) | n=6 (19%) | n=4 (13%) |
| Age at onset (years, median (range)) | 8 (1–50) | 9 (2–21) | 41 (8–61) |
| Age at baseline (years) | 36 (19–54) | 44 (36–51) | 38 (21–64) |
| Maximum CRP (mg/l, median (range)) | 143 (17–330) | 153 (48–350) | 90 (76–220) |
| Amyloidosis (%) | n=9 (43%) | n=3 (50%) | 0 |
| FMF activity score (range 1–14) | 11 (5–14) | 9 (5–12) | 7.5 (5–10) |
| Attacks per year with colchicines | 12 (4–24) | 12 (5–24) | 7 (2–18) |
| Colchicine dose (mg/d) | 2 (0.5–3) | 1.5 (1–2) | 1.5 (0.5–2) |
| Anakinra daily (n=20) | 17 (81%) | 1 (17%) | 2 (50%) |
| Anakinra less than daily (n=9) | 3 (14%) | 4 (66%) | 2 (50%) |
| Canakinumab (n=2) | 1 (5%) | 1 (17%) | 0 |