Table 1 Demographic, clinical and laboratory findings and characteristic patterns of hearing loss in patients with Muckle-Wells syndrome (MWS)
From: Early detection of sensorineural hearing loss in Muckle-Wells-syndrome
| Â | Patients |
|---|---|
N = 23 | |
Demographics | |
 Median age at diagnosis of MWS [years] (range) | 18 (3–72) |
 Children ≤18 years at diagnosis (%) | 12 (52) |
 Females (%) | 15 (65) |
NLRP3 mutation | |
 • c.937G>A (p.Glu313Lys) (%) | 14 (61) |
 • c.1049C>T (p.Thr350Met) (%) | 5 (22) |
 • c.598G>A (p.Val200Met) (%) | 4 (17) |
Overall disease activity | |
 Median MWS-DAS at baseline (range) | 8 (6–16) |
Laboratory markers | |
 Mean SAA at baseline in mg/l (std), (nl* <1) | 6.87 (1.43) |
 Mean CRP at baseline in mg/l (std), (nl* <0.05) | 0.22 (0.17) |
Treatment | |
 IL-1 Inhibition (%) | 23/23 (100) |
 Anakinra (%) | 10 (43) |
 Canakinumab (%) | 13 (57) |