Table 2. PH targeted therapy at inclusion and 1 year after diagnosis.

	IPAH (n = 798)	PH-IIP (n = 151)	p value
Baseline
ERA monotherapy	172 (21.6%)	11 (7.3%)	<0.001
PDE-5 inhibitor monotherapy	461 (57.8%)	133 (88.1%)	<0.001
PCA monotherapy	13 (1.6%)	0	t.n.a.
Other monotherapy	29 (3.6%)	0	t.n.a.
ERA+PDE-5 inhibitor	85 (10.7%)	4 (2.6%)	0.001
Other double combination therapies	29 (3.6%)	3 (2.0%)	0.459
1 year
ERA monotherapy	72 (13.6%)	4 (4.7%)	0.020
PDE-5 inhibitor monotherapy	228 (43.1%)	75 (87.2%)	<0.001
PCA monotherapy	0	1 (1.2%)	t.n.a.
Other monotherapy	8 (1.5%)	0	t.n.a.
ERA+PDE-5 inhibitor	129 (24.4%)	2 (2.3%)	<0.001
Other double combination therapies	42 (7.9%)	0	0.002
Triple combination therapy	33 (6.2%)	0	t.n.a.
No therapy	17 (3.2%)	4 (4.7%)	t.n.a.

Abbreviations: IPAH, idiopathic pulmonary arterial hypertension; PH-IIP, pulmonary hypertension associated with idiopathic interstitial pneumonia; ERA, endothelin receptor antagonist; PDE5, phosphodiesterase-5; PCA, prostacyclin analogue; t.n.a, test not applicable