Patient/parent completed 1 months’ diary score with items dichotomised as 0/1 in 98 patients with hereditary recurrent fever syndromes
| Diary item | FMF (N=39) | CAPS (N=35) | TRAPS (N=14) | MKD (N=10) | Total (N=98) |
|---|---|---|---|---|---|
| (a) Fever ≥38°C (100.4°F) | 2.77±4.27 | 0.94±2.42 | 5.36±9.15 | 2.80±2.04 | 2.35±4.61 |
| (b) Overall symptoms | 5.56±6.43 | 3.97±9.05 | 13.86±20.16 | 7.70±8.21 | 6.86±11.22 |
| (c) Abdominal pain | 6.72±6.66 | 1.57±5.56 | 4.14±7.49 | 3.40±7.66 | 3.90±6.58 |
| (d) Nausea/vomiting | 1.97±4.14 | 0.80±2.84 | 0.00±0.00 | 1.60±1.58 | 1.17±3.10 |
| (e) Diarrhoea | 2.31±5.11 | 0.31±1.69 | 0.14±0.53 | 2.30±4.06 | 1.44±4.04 |
| (f) Headaches | 2.62±3.85 | 5.29±13.81 | 3.79±5.16 | 5.70±6.02 | 3.93±8.66 |
| (g) Chest pain | 0.85±1.81 | 0.14±0.55 | 1.14±3.03 | 0.00±0.00 | 0.52±1.62 |
| (h) Painful nodes | 1.05±3.91 | 0.77±2.21 | 0.93±2.56 | 6.20±5.33 | 1.40±3.58 |
| (i) Arthralgia or myalgia | 3.77±6.08 | 6.11±13.13 | 11.36±18.11 | 3.20±4.71 | 5.37±10.89 |
| (j) Swelling of the joints | 1.23±3.00 | 0.94±2.90 | 2.14±5.57 | 0.00±0.00 | 1.06±3.18 |
| (k) Eye manifestations | 0.26±1.02 | 3.57±7.76 | 3.43±9.20 | 0.30±0.67 | 2.15±6.98 |
| (l) Skin rash | 1.44±6.73 | 3.43±9.37 | 5.00±9.16 | 1.40±2.67 | 3.15±8.87 |
| Total AIDAI score | 19.10±16.41 | 20.49±26.00 | 34.93±49.13 | 22.50±15.93 | 22.20±26.79 |
Data are means±SD, maximum 31 points per item.
AIDAI, Auto-Inflammatory Diseases Activity Index; CAPS, cryopyrin-associated periodic syndromes; FMF, familial Mediterranean fever; MKD, mevalonate kinase deficiency; TRAPS, tumour necrosis factor receptor-associated periodic syndrome.