Table 1.
General patient characteristics
| SScPAH N = 5 |
IPAH N = 9 |
PVOD N = 6 |
Control N = 5 |
|
|---|---|---|---|---|
| Age, yrs | 51 (32 to 60) | 53 (23 to 59) | 33 (23 to 59) | 33 (24 to 76) |
| Male/Female (n) | 1/4 | 2/7 | 3/3 | 5/0 |
| Antibody (Ab) profile | anti-centromere: 5 (100%) |
0 | 0 | |
| Disease duration of PAH at time of death/biopsy (yrs) | 1 (0.1 to 4.0) | 2.6 (0.8 to 9.0) | 1.9 (0.08 to 5.0) | |
| Therapy at time of death/biopsy |
||||
| Monotherapy: | ||||
| prostacycline | 3 | 6 | 4 | |
| PDE-5 inhibitor | 0 | 1 | 0 | |
| ERA | 0 | 1 | 2 | |
| Combination therapy: | ||||
| Prostacycline+ERA+ | 0 | 0 | ||
| PDE-5 inibitor (n) | 1 | |||
| ERA+PDE-5 inhibitor (n) | 1 | 0 | 0 | |
| ERA+prostacycline (n) | 0 | 1 | 0 | |
| Limited cutaneous SSc, n (%) | 5 (100%) | |||
| SSc disease duration, yrs * | 2 (1 to 34) | |||
| Raynaud phenomenon duration, yrs | 13 (1 to 40) |
Values expressed as median (range) or otherwise as stated. * Since first non-Raynaud symptom, at time of diagnosis of pulmonary arterial hypertension.
ABS, atrial balloon septostomia; ERA, endothelin receptor antagonist; IPAH, idiopathic PAH; PDE-5, phosphodiesterase 5; PVOD, pulmonary veno-occlusive disease; SScPAH, systemic sclerosis-associated pulmonary arterial hypertension (PAH).