| NYHA class I/II n = 9 | NYHA class III/IV n = 12 | P-value |
---|---|---|---|
Female sex, n (%) | 9 (100) | 11 (91.7) | 0.375 |
Age at SLE diagnosis | 26.7 ± 10.9 | 30.3 ± 9.9 | 0.219 |
Age at PAH diagnosis | 35.8 ± 11.7 | 35.3 ± 12.3 | 0.922 |
SLE length at PAH diagnosis | 9.1 ± 7.5 | 4.9 ± 4.9 | 0.138 |
Smoking, n (%) | 1 (11) | 0 (0) | 0.352 |
Miscarriage, n (%) | 3 (33) | 3 (25) | 0.676 |
Pregnancy, n (%) | 6 (66.7) | 8 (66.7) | 1.000 |
Raynaud’s phenomenon, n (%) | 7 (77.8) | 10 (81) | 0.748 |
Livedo reticularis, n (%) | 1 (11.1) | 0 (0) | 0.237 |
Arterial thrombosis, n (%) | 0 (0) | 1 (8.3) | 0.375 |
Venous thrombosis, n (%) | 4 (44.4) | 1 (8.3) | 0.055 |
Anti-SM, n (%) | 2 (25) | 2 (22.2) | 0.893 |
Anti-RNP, n (%) | 4 (50) | 7 (77.8) | 0.232 |
Anti-Ro, n (%) | 2 (25) | 3 (33.3) | 0.707 |
Anti-La, n (%) | 0 (0) | 2 (22.2) | 0.156 |
Positive anti- aCL IgM, n (%) | 1 (12.5) | 1 (10) | 0.867 |
Positive anti- aCL IgG, n (%) | 1 (12.5) | 2 (20) | 0.671 |
Positive anti-dsDNA, n (%) | 2 (25) | 1 (11.1) | 0.453 |
C2 (mg/dL) | 85.2 ± 34.3 | 88.1 ± 28.6 | 0.859 |
CH100 (mg/dL) | 83.8 ± 30.2 | 93.1 ± 31.4 | 0.579 |
CRP (mg/dL) | 3.43 ± 2.01 | 19.20 ± 50.20 | 1.000 |
SLEDAI | 2 ± 4.5 | 3.0 ± 3.4 | 0.299 |
6-min walk test | 413 ± 14.8 | 417.7 ± 92.9 | 0.934 |
FVC (%) | 84.1 ± 17.5 | 75.7 ± 13.2 | 0.143 |
Dco (%) | 52.2 ± 8.3 | 44.5 ± 23.2 | 0.548 |
Death, n (%) | 1 (11.1) | 5 (41.7) | 0.125 |
Nailfold capillaroscopy | |||
 SD pattern, n (%) | 4 (66.7) | 5 (50) | 0.515 |
 Normal or unspecific | 2 (33.3) | 5 (50) |  |
RHC | |||
 MPAP | 47.6 ± 7.7 | 50.0 ± 14.6 | 0.654 |
 CI | 2.61 ± 0.66 | 2.36 ± 0.75 | 0.441 |
 PVRI | 8.86 ± 3.0 | 12.62 ± 11.47 | 0.824 |
Treatment | |||
 Sildenafil, n (%) | 4 (44.4) | 8 (67) | 0.309 |
 Bosentan, n (%) | 5 (55.6) | 5 (41.7) | 0.528 |
 Cyclophosphamide IV, n (%) | 4 (44.4) | 6 (50) | 0.801 |
 Methylprednisolone IV, n (%) | 3 (33.3) | 5 (41.7) | 0.697 |
 Oral anticoagulants, n (%) | 6 (66.7) | 7 (63.6) | 0.642 |