Pharmacological management for pulmonary hypertension in systemic sclerosis

Pharmacological management for pulmonary arterial hypertension in systemic sclerosis is highly specialised. Refer all people with suspected pulmonary arterial hypertension to a specialist centre for management.

The following classes of drugs are effective for pulmonary arterial hypertension:

• endothelin receptor antagonists (bosentan, ambrisentan, macitentan)
• phosphodiesterase type 5 (PDE5) inhibitors (sildenafil, tadalafil)
• prostanoid analogues (epoprostenol, iloprost)
• prostacyclin receptor agonists (selexipag)
• soluble guanylate cyclase stimulators (riociguat)
• calcium channel blockers (amlodipine, nifedipine, diltiazem).

See specific therapies for pulmonary hypertension in the Cardiovascular guidelines. The role of anticoagulation in patients with pulmonary arterial hypertension remains uncertain.