Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

Table 2 Independent predictors of mortality in SSc-PAH determined by multivariable Cox proportional hazard regression analysis

Characteristic	Hazard ratio (95% CI)	P value
Age at diagnosis of PAH, years	1.1 (1.0–1.1)	0.03
ILD on HRCT (FVC >60%)	2.8 (1.4–5.6)	0.01
WHO functional class	2.0 (1.1–3.9)	0.03
Pulmonary arterial pressure at PAH diagnosis, mmHg	1.1 (1.0–1.1)	0.001
Digital ulcers present ever	3.1 (1.4–7.2)	0.01
Specific PAH therapies and anticoagulation
Vasodilator monotherapy only	reference	reference
Vasodilator monotherapy and anticoagulation	0.39 (0.1–1.2)	0.09
Vasodilator combination therapy only	0.49 (0.2–1.2)	0.10
Vasodilator combination therapy and anticoagulation	0.28 (0.1–0.7)	0.01

Abbreviations: SSc systemic sclerosis, PAH pulmonary arterial hypertension, WHO world health organization, ILD interstitial lung disease, HRCT high-resolution computer tomography. FVC forced vital capacity, 6MWD six-minute walk distance, mRAP mean right atrial pressure, mPAP mean pulmonary arterial pressure, HCQ hydroxychloroquine

ISSN: 1478-6362