HR of overall mortality across demographic and clinical characteristics among the at-risk patients.
| Characteristics^ | n (%) | HR (95% CI)* | p |
|---|---|---|---|
| Sex | |||
| Female | 207 (88) | 1 (ref) | |
| Male | 29 (12) | 4.72 (1.79–12.45) | 0.0017 |
| Race | |||
| Other | 66 (28) | 1 (ref) | |
| White | 170 (72) | 1.18 (0.41–3.40) | 0.7603 |
| UCSD-DI score | |||
| No difficulty | 109 (48) | 1 (ref) | |
| Difficulty | 116 (52) | 2.32 (0.90–5.99) | 0.0808 |
| SHAQ score | |||
| Normal (< 1) | 138 (61) | 1 (ref) | |
| Abnormal (1–3) | 88 (39) | 1.50 (0.58–3.87) | 0.3995 |
| Pulmonary function test** | |||
| % FVC predicted | 224 | 1.34 (1.03–1.75) | 0.0276 |
| % DLCO predicted | 216 | 2.07 (1.43–3.01) | 0.0001 |
| 6-MWT, m** | 154 | 1.07 (1.01–1.13) | 0.0292 |
| Exercise O2 desaturation (%) | 152 | 5.39 (1.66–17.5) | 0.005 |
| Decline in O2 saturation below 90% during exercise*** | |||
| No | 119 (79) | 1 (ref) | |
| Yes | 32 (21) | 9.53 (2.58–35.20) | 0.0007 |
| HRCT | |||
| Normal HRCT | 43 (26) | 1 (ref) | |
| Fibrosis, ground glass, honeycombing on HRCT | 121 (74) | 2.74 (0.76–9.91) | 0.1251 |
| Echo/Doppler** | |||
| Ejection fraction (%) | 212 | 0.87 (0.54–1.40) | 0.5661 |
| Pericardial effusion | |||
| Without effusion | 163 (89) | 1 (ref) | |
| With effusion | 21 (11) | 5.32 (1.78–15.84) | 0.0027 |
| Mean PAP, mmHg | 61 | 0.58 (0.21–1.60) | 0.292 |
| Right heart catheterization** | |||
| Pulmonary capillary wedge pressure, mmHg | 61 | 1.12 (0.71–1.78) | 0.6199 |
| Cardiac output, l/min | 59 | 1.17 (0.85–1.60) | 0.3392 |
| Pulmonary vascular resistance, dynes-sec/cm5 | 58 | 0.48 (0.28–0.83) | 0.0084 |
| Hemoglobin, g/dl | |||
| ≥ 11.2 | 188 (85) | 1 (ref) | |
| < 11.2 | 33 (15) | 3.76 (1.33–10.63) | 0.0126 |
| Disease type | |||
| Limited | 154 (67) | 1 (ref) | |
| Diffuse | 75 (33) | 1.64 (0.62–4.34) | 0.3223 |
↵^ Characteristics were defined at baseline or within 6 mos prior to PH diagnosis.
↵* HR were derived from separate models for each predictor while adjusting for age, sex, race (white vs nonwhite), and duration since first Raynaud symptoms; with an interaction term between time-dependent PH status and the predictor of interest.
↵** For continuous variables, HR correspond to a decrease in the predictor variable of the specified number of units: % FVC predicted (15%), % DLCO predicted (15%), 6-MWT (15 m), exercise oxygen saturation (15%), ejection fraction (6%), mean PAP (4 mmHg), pulmonary capillary wedge pressure (2 mmHg), cardiac output (0.5 l/min), pulmonary vascular resistance (40 dynes-sec/cm5).
↵*** Defined as participants with O2 saturation below 90% during exercise and above 90% at rest; excludes those with missing 6-MWT and 1 participant with O2 saturation below 90% at rest. 6-MWT: six-minute walk test; UCSD-DI: University of California at San Diego Dyspnea Index; SHAQ: Scleroderma Health Assessment Questionnaire; FVC: forced vital capacity; HRCT: high-resolution computed tomography; PAP: pulmonary arterial pressure; PH: pulmonary hypertension.