Mutational status in MEFV gene of included patients
| Heterozygous (class IV-V/WT and class IV-V/III) | Class IV-V variant | Homozygous (only class IV/V variant) | Compound heterozygous (only class IV/V variant) | Class III | |||||
| Class IV-V variant | n | Additional class III variant | n | n | n | Cass III variant | III/WT n | III/III n | |
| Total | 224 | Total | 57 | Total | 233 | 402 (201 patients) | Total | 49 | 80 (40 patients) |
| M694V | 152 | E148Q | 34 | M694V | 196 | 154 | A744S | 6 | |
| M694I | 14 | P369S | 5 | M694I | 7 | 30 | E148Q | 28 | 42 |
| M680I | 13 | A744S | 6 | M680I | 14 | 73 | K695R | 8 | 1 |
| V726A | 35 | K695R | 8 | V726A | 12 | 104 | I591T | 3 | 2 |
| R761H | 4 | E230K | 1 | R761H | 3 | 26 | R202Q | 2 | 0 |
| M680L | 1 | I641F | 1 | M680L | 0 | 1 | R653H | 1 | 0 |
| Q97K | 1 | R354W | 1 | F479L | 0 | 8 | G196W | 1 | 0 |
| S42R | 1 | I59T | 1 | E167D | 0 | 4 | L110P | 0 | 4 |
| T267I | 1 | M694L | 1 | 0 | P369S | 0 | 20 | ||
| A89T | 1 | I692del | 0 | 1 | R408Q | 0 | 9 | ||
| M694L | 1 | Other | 0 | 2 | Other | 0 | 2 | ||
WT, wild type.