Table 1 Patient characteristics and prevalence of CAPS symptoms
Included in study | |
|---|---|
Number of patients | 24 |
Male gender | 9 (37.5%) |
Age group | |
Child (0–12 years) | 4 (16.7%) |
Adolescent (12–18 years) | 3 (12.5%) |
Adult (18 years or older) | 17 (70.8%) |
Age (years, median (range)) | |
Onset | 5 (0–54) |
Diagnosis | 20 (1–76) |
Start anti-IL-1 | 19 (1–76) |
Start study | 28 (5–82) |
T0 treatment | |
No | 2 (8.3%) |
Anakinra | 3 (12.5%) |
Canakinumab | 19 (79.2%) |
Duration of treatment at T0 (months, median (range)) | |
Anakinra | 46 (42–48) |
Canakinumab | 49 (1–76) |
Dose (mg, median (range))) | |
Anakinra | 100 |
Canakinumab | 150 (45–300) |
Dose interval (median, (range)) | |
Anakinra (days) | 1 |
Canakinumab (weeks) | 8 (4–10) |
Pattern | |
Continuous | 5 (20.8%) |
Episodic | 10 (41.7%) |
Continuous with flares | 9 (37.5%) |
NLRP3 mutation | |
V198 M | 5 (20.8%) |
T348 M | 3 (12.5%) |
Y859H | 3 (12.5%) |
W414 L | 3 (12.5%) |
A439V | 2 (8.3%) |
R488K | 1 (4.2%) |
No mutation | 7 (29.2%) |
Symptoms | |
Fever | 13 (54.2%) |
Skin rash | 20 (83.3%) |
Musculoskeletal complaints | 21 (87.5%) |
Ocular symptoms | 13 (54.2%) |
Neurologic symptoms | 16 (66.7%) |
Gastrointestinal symptoms | 8 (33.3%) |
Lymphoreticular symptoms | 4 (16.7%) |
Cardiopulmonal symptoms | 3 (12.5%) |
Other symptoms | 8 (33.3%) |
Complications | 13 (54.2%) |