|
Age, years
|
61.3 ± 8.2
|
60.9 ± 8.0
|
61.7 ± 8.9
|
NS
|
|
Female sex, n (%)
|
16 (80%)
|
8 (80%)
|
8 (80%)
|
NS
|
|
Disease subtype
|
|
Limited, n (%)
|
16 (80%)
|
8 (80%)
|
8 (80%)
|
NS
|
|
Diffuse, n (%)
|
4 (20%)
|
2 (20%)
|
2 (20%)
|
NS
|
|
Autoantibody subtype, n (%)
|
|
Positive anti-Scl70
|
2 (10%)
|
0
|
2 (20%)
|
NS
|
|
Positive anti-centromere
|
5 (25%)
|
4 (40%)
|
1 (10%)
|
NS
|
|
Positive anti-RNA polymerase
|
0
|
0
|
0
|
–
|
|
Positive ANA, centromere pattern
|
8 (42.1%)
|
5 (50%)
|
3 (33%)
|
NS
|
|
Positive ANA, nucleolar pattern
|
10 (52.6%)
|
4 (40%)
|
6 (67%)
|
NS
|
|
Positive ANA, RNP
|
1 (5.3%)
|
1 (10%)
|
0
|
NS
|
|
Medications (ever-usage)
|
|
Calcium channel blockade, n (%)
|
17 (85%)
|
8 (80%)
|
9 (90%)
|
NS
|
|
Azathioprine, n (%)
|
0
|
0
|
0
|
NS
|
|
Cychophosphamide, n (%)
|
0
|
0
|
0
|
−
|
|
D-Penicillamine, n (%)
|
0
|
0
|
0
|
−
|
|
Intravenous immunoglobulins, n (%)
|
0
|
0
|
0
|
−
|
|
Leflunomide, n (%)
|
0
|
0
|
0
|
−
|
|
Methotrexate, n (%)
|
0
|
0
|
0
|
−
|
|
Mycophenolate mofetil, n (%)
|
5 (25%)
|
1 (10%)
|
4 (40%)
|
0.04
|
|
Plaquenil, n (%)
|
6 (30%)
|
4 (40%)
|
2 (20%)
|
NS
|
|
Predisone, n (%)
|
6 (31.6%)
|
2 (22%)
|
4 (40%)
|
NS
|
|
Rituximab, n (%)
|
0
|
0
|
0
|
−
|
|
PAH Therapies (ever-usage)
|
|
Endothelin receptor antagonist, n (%)
|
6 (31.6%)
|
1 (11.1%)
|
5 (50%)
|
NS
|
|
Phosphodiesterase type 5 inhibitors, n (%)
|
9 (47.4%)
|
1 (11.1%)
|
8 (80%)
|
0.001
|
|
Prostacyclin analogs/receptor agonists, n (%)
|
3 (15.8%)
|
1 (11.1%)
|
2 (20%)
|
NS
|
|
WHO FC I/II/III, n
|
11/6/3
|
9/1/0
|
2/5/3
|
0.02
|
|
FVC, %
|
86 ± 21
|
92 ± 14
|
79 ± 26
|
NS
|
|
6-min walking distance, m
|
NA
|
NA
|
397 ± 122
|
−
|
|
Right atrial pressure, mmHg
|
NA
|
NA
|
5 ± 4
|
−
|
|
Mean PAP, mmHg
|
NA
|
NA
|
36 ± 7
|
−
|
|
PCWP, mmHg
|
NA
|
NA
|
9 ± 4
|
−
|
|
Cardiac Index, l/min/m2
|
NA
|
NA
|
2.3 ± 0.7
|
−
|
|
PVR, WU
|
NA
|
NA
|
6.1 ± 2.7
|
−
|
