Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis

Table 3 Multivariable model for survival from disease onset to death by ILD severity

Disease characteristic	HR	95% CI	p-value
Age at SSc onset (years)	1.11	1.09–1.12	< 0.001
ANA centromere positive	0.65	0.48–0.91	0.011
Female sex	0.61	0.43–0.86	0.005
History of renal crisis	2.60	1.48–4.56	0.001
ILD-only (limited)^a	1.52	0.96–2.42	0.074
ILD-only (extensive)^a	3.72	2.26–6.13	< 0.001
PAH	4.30	2.95–6.27	< 0.001
PAH and limited ILD^a	2.60	1.59–4.24	< 0.001
PAH and extensive ILD^a	5.68	3.51–9.17	< 0.001
Charlson Comorbidity Index Score	0.94	0.86–1.02	0.156

Disease onset is defined as the onset of the first non-Raynaud’s phenomenon symptom
Abbreviations: ANA anti-nuclear antibody, ILD interstitial lung disease, PAH pulmonary arterial hypertension
^aILD severity defined as limited if < 20% involvement on HRCT or 20–30% HRCT involvement with FVC ≥ 70%, or extensive if > 30% HRCT involvement, or 20–30% HRCT involvement and FVC < 70%

ISSN: 1478-6362