Data | Number | Familial cases | Sporadic cases | Univariate model raw p value* | Multivariate model p value |
---|---|---|---|---|---|
Epidemiological data | |||||
N | 463 | 207 (44.7%) | 256 (55.3%) | ||
Sex | 463 | 0.49 | |||
Male | 201 | 86 (41.6%) | 115 (44.9%) | 0.20 | |
Female | 262 | 121 (58.4%) | 141 (55.1%) | Ref. | |
Age at diagnosis | 424 | 0.001 [0.021] | 0.016 | ||
Mean ± SD | 38.2 ± 13.1 | 43.0 ± 15.5 | |||
Median [q1; q3] | 35.5 [29.3; 46.2] | 40.3 [30.6; 54.0] | |||
Ethnicity | 303 | 0.85 | |||
European | 235 | 112 (77.2%) | 123 (77.9%) | ||
Caribbean or Sub-Saharan African | 68 | 33 (22.8%) | 35 (22.1%) | ||
Smokers | 381 | 0.43 | |||
Yes | 156 | 56 (38.4%) | 100 (42.6%) | ||
No | 225 | 90 (61.6%) | 135 (57.4%) | ||
Clinical data at diagnosis | |||||
Disease onset | 401 | 0.015 [0.28] | |||
Asymptomatic | 137 | 45 (27.3%) | 92 (39.0%) | ||
Symptomatic | 264 | 120 (72.7%) | 144 (61.0%) | ||
Löfgren syndrome | 378 | 0.26 | |||
Yes | 58 | 29 (17.8%) | 29 (13.5%) | ||
No | 320 | 134 (82.2%) | 186 (86.5%) | ||
Number of organs involved | 414 | 0.144 | |||
1 | 158 | 69 (39.9%) | 89 (36.9%) | ||
2 | 117 | 36 (20.8%) | 81 (33.6%) | ||
3 | 70 | 29 (16.8%) | 41 (17.0%) | ||
≥ 4 | 69 | 39 (22.5%) | 30 (12.5%) | ||
Organs involved | 414 | <0.001 [0.003] | |||
Lungs only | 148 | 68 (39.31%) | 80 (33.2%) | 0.65 | |
Lungs and Eyes | 53 | 10 (5.78%) | 43 (17.84%) | 0.076 | |
Lungs and Skin | 21 | 15 (8.67%) | 6 (2.49%) | 0.041 | |
Other organs | 192 | 80 (46.24%) | 112 (46.47%) | Ref | |
X-ray staging | 456 | 0.20 | 0.78 | ||
0 | 68 | 40 (19.8%) | 28 (11.0%) | ||
1 | 162 | 66 (32.7%) | 96 (37.8%) | ||
2 | 157 | 69 (34.2%) | 88 (34.7%) | ||
3 | 48 | 15 (7.4%) | 33 (13.0%) | ||
4 | 21 | 12 (5.9%) | 9 (3.5%) | ||
PFT at first visit | |||||
TLC (%) | 215 | 0.93 | |||
< 80% | 47 | 18 (22.2%) | 29 (21.6%) | ||
≥ 80% | 168 | 63 (77.8%) | 105 (78.4%) | ||
FEV1/FVC | 242 | 0.43 | |||
< 70% | 55 | 18 (20.0%) | 37 (24.3%) | ||
≥ 70% | 187 | 72 (80.0%) | 115 (75.7%) | ||
Biology at first visit | |||||
Bronchoalveolar lavage lymphocytes (%) | 95 | 0.171 | |||
< 10 | 23 | 3 (11.6%) | 20 (29.0%) | ||
[10; 20] | 17 | 5 (19.2%) | 12 (17.4%) | ||
> 20 | 55 | 18 (69.2%) | 37 (53.6%) | ||
Calcemia | 256 | 0.80 | |||
< 2.25 | 52 | 21 (22.6%) | 31 (19.0%) | ||
[2.25 - 2.5] | 182 | 64 (68.8%) | 118 (72.4%) | ||
> 2.5 | 22 | 8 (8.6%) | 14 (8.6%) | ||
Treatment | |||||
Treatment | 463 | <0.001 [0.008] | |||
None | 136 | 79 (38.2%) | 57 (22.3%) | ||
At least one | 327 | 128 (61.8%) | 199 (77.7%) | ||
Type of treatment | 327 | 0.188 | |||
Corticosteroids only | 186 | 67 (52.3%) | 119 (59.8%) | ||
Other treatment | 141 | 61 (47.7%) | 80 (40.2%) | ||
Pattern of disease course | |||||
SCAC | 414 | 0.003 [0.0497] | |||
1- symptomatic - no treatment | 48 | 30 (18.1%) | 18 (7.3%) | ||
2- symptomatic - treatment ≤ 12 mo. | 47 | 18 (10.8%) | 29 (11.7%) | ||
3- symptomatic - treatment > 12 mo. | 172 | 72 (43.4%) | 100 (40.3%) | ||
4- asymptomatic - no treatment | 51 | 19 (11.5%) | 32 (12.9%) | ||
5- asymptomatic - treatment ≤ 12 mo. | 16 | 7 (4.2%) | 9 (3.6%) | ||
6- asymptomatic - treatment > 12 mo. | 80 | 20 (12.0%) | 60 (24.2%) | ||
Classification of outcome | 138 | 0.165** | |||
1- recovery within 3 years | 72 | 24 (57.14%) | 48 (50%) | ||
2- recovery between 3 and 5 years | 32 | 6 (14.29%) | 26 (27.08%) | ||
3- no recovery at 5 years | 30 | 12 (28.57%) | 18 (18.75%) | ||
4- death | 4 | 0 (0%) | 4 (4.17%) | ||
Genetic data | |||||
BTNL2 | 436 | 0.17*** | 0.39*** | ||
BTNL2 A/A | 201 | 89 (47.8%) | 112 (44.8%) | ||
BTNL2 A/G | 194 | 84 (45.2%) | 110 (44.0%) | ||
BTNL2 G/G | 41 | 13 (7.0%) | 28 (11.2%) |