Table 2.
FVC ≤ 79% DLCO ≤ 79% 16 patients |
FVC ≥ 80% DLCO ≤ 79% 74 patients |
FVC ≥ 80% DLCO ≥ 80% 10 patients |
|
Age (years), mean ± sd | 55.1 ± 12.4 | 56.1 ± 11.9 | 50.7 ± 11.4 |
Disease duration (years), median (I.Q. range) | 7 (4 – 18) | 6 (3 – 13) | 4.5 (1 – 14.5) |
Sex n (%) | 14 F (87.5%) 2 M (12.5%) |
68 F (91.9%) 6 M (8.1%) |
10 F (100.0%) 0 M (0.0%) |
Skin involvement: | |||
dSSc n (%) | 12 (75.0%) | 12 (16.2%) | 0 (0.0%) |
lSSc n (%) | 4 (25.0%) | 62 (83.8%) | 10 (100.0%) |
Skin score mean ± sd | 14.8 ± 10.7 | 9.9 ± 7.4 | 6.7 ± 4.6 |
Autoantibodies pattern: | |||
ACA n (%) | 2 (12.5%) | 32 (43.2%) | 7 (70.0%) |
AntiScl 70 n (%) | 11 (68.7%) | 25 (33.8%) | 2 (20.0%) |
Antinucleolus n (%) | 0 (0.0%) | 9 (12.2%) | 1 (10.0%) |
AntiRNP n (%) | 1 (6.3%) | 5 (6.8%) | 0 (0.0%) |
ACA/Scl 70/nucleolus/RNP negative n (%) | 2 (12.5%) | 3 (4.0%) | 0 (0.0%) |
FVC (%) mean ± sd | 65.0 ± 8.8 | 104.8 ± 16.3 | 122.0 ± 13.3 |
≤ 69% n (%) | 12 (75.0%) | 0 (0.0%) | 0 (0.0%) |
≤ 49% n (%) | 1 (6.3%) | 0 (0.0%) | 0 (0.0%) |
DLCO (%) mean ± sd | 39.8 ± 11.8 | 59.0 ± 10.4 | 94.2 ± 12.6 |
≤ 69% n (%) | 16 (100%) | 67 (90.5%) | 0 (0.0%) |
≤ 49% n (%) | 13 (81.3%) | 16 (21.6%) | 0 (0.0%) |
FVC and DLCO ≤ 69% | 12 (75.0%) | 0 (0.0%) | 0 (0.0%) |
FVC and DLCO ≤ 49% | 1 (6.3%) | 0 (0.0%) | 0 (0.0%) |
PASP ≥ 40 mmHg n (%) | 6 (37.5%) | 5 (6.7%) | 0 (0.0%) |
≥ 60 mmHg n (%) | 1 (6.3%) | 1 (1.3%) | 0 (0.0%) |
PFTs: pulmonary function tests; FVC: forced vital capacity; DLCO: diffusing capacity for carbon monoxide; dSSc: diffuse skin involvement; lSSc: limited skin involvement; ACA: anticentromere antibodies; antiScl 70: antitopoisomerasi I antibodies; antiRNP: antiribonucleoproteins antibodies; PASP: pulmonary artery systolic pressure.