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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.



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.
Raynaud’s section
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.
Act on blood vessels Headache, muscle pain, flushing, stomach upset, nausea, respiratory tract infection, nasal stuffiness.


Scleroderma Medications Guide (Canada)