Platelet-derived growth factor receptor-β and epidermal growth factor receptor in pulmonary vasculature of systemic sclerosis-associated pulmonary arterial hypertension versus idiopathic pulmonary arterial hypertension and pulmonary veno-occlusive disease: a case-control study

Table 1 General patient characteristics

	SScPAH N= 5	IPAH N= 9	PVOD N= 6	Control N= 5
Age, yrs	51 (32 to 60)	53 (23 to 59)	33 (23 to 59)	33 (24 to 76)
Male/Female (n)	1/4	2/7	3/3	5/0
Antibody (Ab) profile	anti-centromere: 5 (100%)	0	0
Disease duration of PAH at time of death/biopsy (yrs)	1 (0.1 to 4.0)	2.6 (0.8 to 9.0)	1.9 (0.08 to 5.0)
Therapy at time of death/biopsy
Monotherapy:
prostacycline	3	6	4
PDE-5 inhibitor	0	1	0
ERA	0	1	2
Combination therapy:
Prostacycline+ERA+		0	0
PDE-5 inibitor (n)	1
ERA+PDE-5 inhibitor (n)	1	0	0
ERA+prostacycline (n)	0	1	0
Limited cutaneous SSc, n (%)	5 (100%)
SSc disease duration, yrs *	2 (1 to 34)
Raynaud phenomenon duration, yrs	13 (1 to 40)

Values expressed as median (range) or otherwise as stated. * Since first non-Raynaud symptom, at time of diagnosis of pulmonary arterial hypertension.
ABS, atrial balloon septostomia; ERA, endothelin receptor antagonist; IPAH, idiopathic PAH; PDE-5, phosphodiesterase 5; PVOD, pulmonary veno-occlusive disease; SScPAH, systemic sclerosis-associated pulmonary arterial hypertension (PAH).

ISSN: 1478-6362