Pulmonary Arterial Hypertension

Symptoms and Treatments

Patients diagnosed with pulmonary arterial hypertension should be referred to a pulmonary hypertension center for experienced evaluation and management of this serious scleroderma complication.

	Action	Most Common Side Effects

Endothelin Receptor Antagonists
Ambrisentan (Volibris®)	Act on blood vessels	Peripheral edema, headache, decreased hemoglobin, nasal stuffiness, palpitations.

Bosentan (Tracleer®)	Act on blood vessels	Headache, nasal stuffiness, flushing, liver function abnormality, flushing, pulmonary edema, low blood pressure.

Macitan (Opsumit®)	Act on blood vessels	Nasal stuffiness, headache, anemia, bronchitis, sore throat, flu.

Prostaglandin Derivatives

Epoprostenol (Flolan®, Caripul®)	Act on blood vessels by inhibiting platelet aggregation	Headache, dizziness, flushing, jaw pain, diarrhea, bone pain, potential serious infection associated with central line catheter in the chest wall.

Trepostinil (Remodulin®)	Act on blood vessels	Pain at infusion site, headache, cough, diarrhea, nausea, flushing, jaw pain, skin rash, edema.

Calcium Channel Blockers

Can be helpful to a small proportion of patients; see Raynaud’s section.	See Raynaud’s section	See Raynaud’s section

Phosphodiesterase Type 5 (PDE5) Inhibitor

Sildenafil citrate (Revatio®)	Act on blood vessels	Headache, flushing, dyspepsia, flushing, nosebleeds, vision change, nasal stuffiness, diarrhea, insomnia.

Tadalafil (Adcirca®)	Act on blood vessels	Headache, muscle pain, flushing, stomach upset, nausea, respiratory tract infection, nasal stuffiness.