All patients (n = 63) | PAH patients (n = 35) | No PH patients (n = 28) | P value | |
---|---|---|---|---|
Femalea | 59 (93.7) | 34 (97.1) | 25 (89.3) | 0.31 |
Age, yearsb | 62.4 (±11.6) | 64.4 (± 10.8) | 59.9 (± 12.4) | 0.13 |
DcSSca | 10 (15.9) | 4 (11.4) | 6 (21.4) | 0.31 |
Time from first symptom, yearsb | 18.6 (± 12.3) | 19.6 (±11.2) | 17.2 (± 13.7) | 0.29 |
Time from first non-RP symptom, yearsb | 10.5 (± 8.9) | 9.7 (±9.4) | 11.5 (± 8.2) | 0.21 |
ACR/EULAR 2013 classification criteriaa | 61 (96.8) | 34 (97.1) | 27 (96.4) | 1.0 |
Telangiectasiasa | 59 (93.7) | 33 (94.3) | 26 (92.9) | 1.0 |
Digital ulcersa | 35 (55.6) | 20 (57.1) | 15 (53.6) | 0.77 |
Scleroderma renal crisisa | 2 (3.2) | 2 (5.7) | 0 (0) | 0.49 |
Interstitial lung diseasea | 32 (50.8) | 19 (54.3) | 12 (42.9) | 0.45 |
Cardiac involvementa | 26 (41.3) | 17 (48.6) | 9 (32.1) | 0.18 |
Antinuclear antibodiesa | 62 (98.4) | 34 (97.1) | 28 (100) | 1.0 |
ACAa | 34 (54.0) | 23 (65.7) | 11 (39.3) | 0.03 |
Anti-Scl-70a | 12 (19.0) | 3 (8.6) | 9 (32.1) | 0.01 |
Nucleolar IIF patterna | 4 (6.3) | 3 (8.6) | 1 (3.6) | 0.62 |
Arterial hypertensiona | 27 (42.9) | 14 (40) | 13 (43.4) | 0.60 |
Dyslipidaemiaa | 15 (23.8) | 6 (17.1) | 9 (32.1) | 0.16 |
Diabetes mellitusa | 0 (0) | 0 (0) | 0 (0) | NA |
Immunosuppressant therapya | 25 (39.7) | 8 (22.9) | 17 (60.7) | <0.01 |
Prednisonea | 19 (30.2) | 7 (20.0) | 12 (40.9) | 0.04 |
Sodium mycophenolatea | 7 (11.1) | 1 (2.9) | 6 (21.4) | 0.03 |
Azathioprine | 3 (4.8) | 0 (0) | 3 (10.7) | 0.08 |
IV cyclophosphamidea | 12 (19.0) | 3 (8.6) | 9 (32.1) | 0.01 |
Calcium channel blockera | 33 (52.4) | 14 (40.0) | 19 (67.9) | 0.02 |
Specific vasodilator therapya | 13 (20.6) | 7 (20.0) | 6 (21.4) | 0.88 |
IV prostanoidsa | 1 (1.6) | 1 (2.9) | 0 (0) | 1.0 |
ERAa | 10 (15.9) | 5 (14.3) | 5 (17.9) | 0.74 |
PDE5 Ia | 5 (7.9) | 2 (5.7) | 3 (10.7) | 0.64 |