Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme

Table 2 Characteristics of patients with SSc by disease subset

	Limited	Diffuse	P value
	n = 1122	n = 377
	mean ± SD or %	mean ± SD or %
Age at recruitment, years	58.8 ± 12.2	53.1 ± 13.1	<0.001
Female	89.5%	74.9%	<0.001
Disease duration^a (non-Raynaud) at recruitment, years	12.2 ± 10.8	8.1 ± 8.7	<0.001
Anti-centromere pattern ANA	59.2%	9.6%	<0.001
Scl 70 positive	8.9%	31.1%	<0.001
RNA polymerase III positive	5.4%	39.6%	<0.001
ILD (HRCT scan)	236 (21.11%)	154 (40.9%)	<0.001
PAH (RHC)	142 (12.7%)	38 (10.1%)	0.40
Rodnan skin score (highest ever)	7.7 ± 5.4	22.7 ± 9.9	<0.001
Digital ulcers ever	39.6%	60.8%	<0.001
Joint contractures ever	28.5%	71.2%	<0.001
Renal crisis ever	0.9%	7.7%	<0.001
Gastro-oesophageal reflux	80.3%	82.7%	0.29
Anal incontinence	27.9%	25.6%	0.39

ANA antinuclear antibodies, ILD interstitial lung disease, HRCT high-resolution chest computed tomography, PAH pulmonary arterial hypertension, RHC right-heart catheterisation
^aDisease duration defined as from first non-Raynaud’s disease manifestation. Disease manifestations are defined as present if ever present from time of diagnosis of systemic sclerosis (SSc)