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Table 1

From: β-Confident-registry: aiming to be largest-ever studied cohort of cryopyrin-associated periodic syndromes (CAPS) patients. Study design and baseline characteristics

  FCAS (n=10) MWS (n=37) NOMID (n=6) Other (n-5)
Age <4 years 0 2 1 1
Age 4- <18 years 2 6 3 1
Age ≥18 years 8 29 3 3
Male, n (%) 3 (30.0) 20 (54.1) 3 (50.0) 2 (40.0)
NLRP3 mutation, n (%) 10 (100.0) 35 (94.6) 5 (83.3) 2 (86.7)
Disease duration (months, mean) 550 370 172 64
Rash/arthralgia/headache/conjunctivitis (%) 100/100/50/80 97.3/97.3/78.4/75.7 100/100/93.4/66.6 80/100/20/40
History of anaemia (%) 10 8.1 50 0
Prior SAA value (mean, mg/L) 37 26 47 3
Prior IL-1 inhibitor treatment, n (%) 0 14 (37.8) 1 (16.1) 2 (40)