Characteristics^ | N | HR (95% CI)* | p |
---|---|---|---|
Sex | |||
Male | 29 (12) | 1 (ref) | |
Female | 207 (88) | 0.80 (0.08–7.74) | 0.844 |
Race | |||
Other | 66 (28) | 1 (ref) | |
White | 170 (72) | 0.67 (0.14–3.31) | 0.6248 |
UCSD-DI score | |||
No difficulty | 109 (48) | 1 (ref) | |
Difficulty | 116 (52) | 2.29 (0.55–9.47) | 0.2537 |
SHAQ score | |||
Normal (< 1) | 138 (61) | 1 (ref) | |
Abnormal (1–3) | 88 (39) | 3.62 (0.86–15.32) | 0.0802 |
Pulmonary function test** | |||
% FVC predicted | 224 | 1.30 (0.86–1.94) | 0.2237 |
% DLCO predicted | 216 | 2.14 (1.14–4.02) | 0.1372 |
FVC change (% change per year) | 127 | 1.55 (1.01–2.38) | 0.039 |
DLCO change (% change per year) | 116 | 1.24 (0.62–2.48) | 0.6775 |
6-MWT distance, m** | 154 | 1.08 (0.98–1.18) | 0.0806 |
Exercise oxygen desaturation (%)*** | 152 | 1.23 (0.13–11.5) | 0.5101 |
Decline in O2 saturation below 90% during exercise*** | |||
No | 119 (79) | 1 (ref) | |
Yes | 32 (21) | 1.20 (0.10–14.54) | 0.2565 |
HRCT | |||
Normal HRCT | 43 (26) | 1 (ref) | |
Fibrosis, ground glass, honeycombing on HRCT | 121 (74) | 0.41 (0.09–1.92) | 0.2565 |
Echo/Doppler** | |||
Ejection fraction (%) | 212 | 1.21 (0.63–2.32) | 0.8566 |
Pericardial effusion | |||
Without effusion | 163 (89) | 1 (ref) | |
With effusion | 21 (11) | 3.19 (0.46–22.03) | 0.2397 |
Hemoglobin, g/dl | |||
≥ 11.2 | 188 (85) | 1 (ref) | |
< 11.2 | 33 (15) | 0.85 (0.09–7.71) | 0.8847 |
Disease type | |||
Limited | 154 (67) | 1 (ref) | |
Diffuse | 75 (33) | 0.88 (0.16–4.78) | 0.883 |
↵^ Defined at baseline or within 6 months 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%), FVC change (6% decrease per year), DLCO change (6% decrease per year), ejection fraction (6% change).
↵*** Defined as participants with O2 saturation below 90% during exercise and above 90% at rest; excludes those with missing 6-minute walk test and 1 participant with O2 saturation below 90% at rest. 6-MWT: 6-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; PH: pulmonary hypertension.