Table 1.
Characteristics of patients recruited up to July 2016 (n = 1636)
| Characteristic | Number (%) or mean ± SD |
|---|---|
| Patient number | 1636 |
| Gender (female:male) | 6.4:1 (86.1% vs 13.9%) |
| Race | |
| Caucasian | 1390 (91.9%) |
| Asian | 75 (4.9%) |
| Aboriginal-Islander | 18 (1.2%) |
| Hispanic | 11 (0.7%) |
| Other | 19 (1.3%) |
| Age at recruitment, mean ± SD, years | 57.2 ± 12.8 |
| Disease duration at recruitmenta, years | 10.9 ± 10.2 |
| Recruited within 4 years of first non-Raynaud’s feature | 435 |
| Age at diagnosis of PAH if present, years | 62.9 ± 11.1 |
| Number of study visits | |
| One | 364 (22.3%) |
| Two | 325 (19.9%) |
| Three | 216 (13.2%) |
| Four | 199 (12.2%) |
| Five | 165 (10.1%) |
| Six | 119 (7.3%) |
| Seven | 71 (4.3%) |
| Eight | 79 (4.8%) |
| Nine | 84(5.2%) |
| Ten | 14 (0.9%) |
| Duration of follow up, mean ± SD, years | 3.7 ± 2.7 |
| Patient status | |
| Current | 1243 (76.5%) |
| Withdrawn | 101 (6.2%) |
| Dead | 220 (13.5%) |
| Lost to follow up | 61 (3.8%) |
| Disease subtype | |
| Limited | 1122 (68.6%) |
| Diffuse | 377 (23.0%) |
| MCTD | 83 (5.3%) |
| Autoantibody profile | |
| ANA positive (n = 1508) | 1418 (94.0%) |
| Anti-centromere (n = 1497) | 673 (44.9%) |
| Anti-Scl70 (n = 1483) | 205 (13.8%) |
| Anti-RNAP (n = 794) | 125 (13.1%) |
aDisease duration from first non-Raynaud manifestation. Numbers of variables analysed (n =) are included in each section of the table to acknowledge any missing data. Patient status: current patients were defined as being seen in the last 2 years, withdrawn patients have withdrawn their consent from participating in the database and lost to follow up is defined as patients who, despite multiple attempts, we have been unable to contact for >18 months. PAH pulmonary arterial hypertension, MCTD mixed connective tissue disease, ANA antinuclear antibody, Anti-RNAP anti-RNA polymerase