Table 1.
Disease characteristics and demographics in patients with PAH-CTD at PATENT-1 baseline
| Characteristic | Overall PAH-CTD (n=111) |
PAH-SSc (n=66) |
PAH-other defined CTD (n=39) |
|---|---|---|---|
| Age, mean±SD (years) | 57±14 | 63±11 | 50±14 |
| Female, n (%) | 98 (88) | 57 (86) | 36 (92) |
| Time from first diagnosis of PH, mean±SD (months) | 27±36 | 26±35 | 26±38 |
| 6MWD, mean±SD (m) | 352±75 | 340±76 | 364±74 |
| WHO FC I/II/III/IV (%)* | 5/36/57/2† | 2/35/59/3† | 10/36/54/0 |
| PAH-specific pretreatment, n (%) | 70 (63) | 45 (68) | 23 (59) |
| Pretreated with ERA | 59 (53) | 39 (59) | 19 (49) |
| Pretreated with PCA | 10 (9) | 6 (9) | 3 (8) |
| Pretreated with ERA and PCA | 1 (1) | 0 | 1 (3) |
| Immunomodulating pretreatment, n (%)‡ | 27 (24) | 16 (24) | 10 (26) |
| Pretreated with immunosuppressive agents§ | 21 (19) | 13 (20) | 8 (21) |
| Pretreated with antineoplastic agents¶ | 14 (13) | 6 (9) | 7 (18) |
| Pretreated with endocrine therapy** | 1 (1) | 1 (2) | 0 |
| Pretreated with other immunomodulating agents | 1 (1) | 0 | 1 (3) |
*Not all percentages add up to 100 due to rounding.
†Data missing for one patient.
‡Non-steroidal anti-inflammatory drugs or disease-modifying antirheumatic drugs.
§Azathioprine, leflunomide, methotrexate, mizoribine, mycophenolate mofetil, mycophenolate sodium, tacrolimus.
¶Celecoxib, cyclophosphamide, methotrexate.
**Estradiol valerate.
6MWD, 6-minute walking distance; CTD, connective tissue disease; ERA, endothelin receptor antagonist; PAH, pulmonary arterial hypertension; PCA, prostacyclin analogue; PH, pulmonary hypertension; SSc, systemic sclerosis; WHO FC, WHO functional class.