Pulmonary arterial hypertension associated with systemic sclerosis: Current diagnostic approach and therapeutic strategies

Table 1 Recommendations for the treatment of systemic sclerosis

Type of drugs	Drugs and doses	Study	Results	Strength of recommendation	Ref.
Endothelin receptor antagonists	Bosentan, 62.5 mg twice daily for 4 wk, followed 125 twice daily for 12 wk	2 RCT	Improves exercise capacity, functional class and some hemodynamic measures	A/B	[56,57]
	Sitaxentan, 100 mg/d for 18 wk	STRIDE-2 study group STRIDE-1 study group	Improves exercise capacity, functional class and some hemodynamic measures	A/B	[64,66]
PDEI-5	Sildenafil, 20, 40, 80 mg three times daily for 12 wk	SUPER study group	Improves exercise capacity, functional class and some hemodynamic measures	A/B	[67]
Prostacyclin analogues	Intravenous epoprostenol at the start usually < 2 ng/kg of body weight per minute (infused continuously by infusion pump); during 12 wk study, doses were adjusted with mean epoprostenol infusion rate of 11 ng/kg per minute	RCT	Improves exercise capacity, functional class and hemodynamic measures	A/B	[46]

RCT: Randomized controlled trials; SUPER: Sildenafil use in pulmonary arterial hypertension; STRIDE: Sitaxsentan to relieve impaired exercise; A/B: Based on studies with high levels of recommendation; PDEI-5: Phosphodiesterase type 5 inhibitor.