Clinical and genetic spectrum of 14 cases of NLRP3-associated autoinflammatory disease (NLRP3-AID) in China and a review of the literature

Table 4 Comparison of clinical manifestations of CAPS among Chinese patients, Japanese patients, and western patients

	Total	Japan	p1 value	Western	p2 value
n	42	19		136
Origin	Chinese	Japanese		Most Caucasian
Gender Ratio	26:16	11:8	0.767^a	69:67	0.205^a
Fever	41 (97.6%)	14 (73.7%)	0.009^b	108 (79%)	0.005^a
Rash	39 (92.9%)	19 (100%)	0.545^b	132 (97%)	0.358^b
Ocular manifestations	15 (35.7%)	12 (63.2%)	0.046^a	97 (71%)	< 0.001^a
Hearing loss	15 (35.7%)	7 (36.8%)	0.932^a	56 (41%)	0.527^a
Neurological symptoms	21/35 (60%)^†	NA		55 (40%)	0.038^a
Severe Neurological symptoms	6/20 (30%)^‡	NA		16 (12%)	0.040^b
Musculoskeletal manifestations	28 (66.7%)	13 (68.4%)	0.892^a	117 (86%)	0.005^a
Severe Musculoskeletal manifestations	4/20 (20.0%)^‡	NA		6 (4%)	0.025^b

^aCompared by Chi-square test, ^bCompared by Fisher exact test
^†Reference [13] didn’t describe the CNS symptoms and thus 7 patients in their cohort were removed in the comparison of neurological symptoms
^‡References [12, 13] didn’t describe the detailed manifestations of CNS and musculoskeletal system, and thus patients in their cohort (15 and 7, respectively) were removed when comparison was performed in terms of severe symptoms
p1 refers Chinese population versus Japanese population, p2 refers Chinese population versus western population

ISSN: 1750-1172