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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.

 

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.

 

Scleroderma Medications Guide (Canada)