Table 1 Main clinical characteristics of patients.
| Mutation characteristics | Total | Group A (R92Q) n (%) | p Value (group A v group B) | Group B (other mutations) n (%) | Group C (P46L) n (%) | p Value (group B v group C) |
|---|---|---|---|---|---|---|
| Patients | 84 | 34 | — | 37 | 13 | — |
| Sex | ||||||
| M | 37 | 17 | >0.99 | 16 | 4 | >0.99 |
| F | 47 | 17 | 21 | 9 | ||
| Ethnicity | 56 C | 28 C | 27 C | 1 C | ||
| 16 MA | 3 MA | 4 MA | 9 MA | <0.001 | ||
| 8 ME | 2 ME | >0.99 | 5 ME | 1 ME | ||
| 2 NA | 1 NA | 0 NA | 2 NA | |||
| 1 I | 0 I | 1 I | 0 I | |||
| Fever | 49 | 16 (48) | 0.128 | 26 (72) | 7 (46) | 0.30 |
| Abdominal pain | 45 | 12 (38.7) | 0.028 | 24 (66) | 9 (70) | <0.99 |
| Musculoskeletal involvement* | 44 | 15 (48) | 0.33 | 22 (61) | 7 (54) | 0.745 |
| Cutaneous involvement† | 28 | 11 (35.5) | 0.99 | 13 (36) | 4 (35) | >0.99 |
| Seritis‡ | 23 | 10 (32) | 0.59 | 9 (25) | 4 (35) | 0.96 |
| Neurological involvement§ | 22 | 5 (16) | 0.39 | 10 (27.7) | 7 (54) | 0.172 |
| Transit disorders¶ | 18 | 7 (22.6) | 0.99 | 8 (22.2) | 3 (35) | >0.99 |
| Lymphadenopathy | 14 | 6 (19) | 0.13 | 2 (5) | 6 (46) | 0.003 |
| Rhinolaryngeal involvement** | 9 | 4 (12) | 0.695 | 3 (8.3) | 2 (15.4) | 0.59 |
| Amyloidosis | 8 | 2 (6.4) | 0.27 | 6 (16.6) | 0 (0) | 0.175 |
| Periocular oedema | 8 | 4 (12) | 0.695 | 3 (8.3) | 1 (7.7) | >0.99 |
| Ocular involvement†† | 5 | 2 (6.4) | 0.99 | 3 (8.3) | 0 (0) | 0.652 |
| Mean age at the first episode (years) | 15.705 | 18.91 | 0.01 | 9.72 | 22.4 | 0.179 |
| Mean duration of episodes (days) | 9.055 | 7.43 | 0.01 | 11.03 | 7.58 | 0.125 |
| Frequency of episodes | ||||||
| ⩽1episode/month | 46 | 13 | 0.017 | 27 | 6 | 0.418 |
| >1episode/month | 27 | 15 | 8 | 4 | ||
| Not related | 11 | 6 | 2 | 3 | ||
| Familial case of TRAPS | 36 | 7 (20.6) | <0.001 | 29 (78.4) | 0 (0) | <0.001 |
| Sporadic case of TRAPS | 48 | 27 | 8 | 13 |
C, Caucasian; I, Indian; MA, Maghrebian; ME, Mediterranean; NA, native African.
*Arthralgia and myalgia; †rash or urticaria or erythema; ‡pleurisy and pericarditis; §headache and aseptic meningitis; ¶constipation or diarrhoea; **pharyngitis; ††conjunctivitis. Comparisons are made between groups A and B, and between groups B and C.