How is pulmonary arterial hypertension (PAH) in scleroderma treated?

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Numerous newer agents have been approved by the FDA for the treatment of pulmonary arterial hypertension (PAH). These include the following:

  • Prostaglandin derivatives such as epoprostenol, treprostinil, beraprost, and iloprost

  • Phosphodiesterase type 5 (PDE-5) inhibitors such as sildenafil and tadalafil

  • Endothelin receptor antagonists such as bosentan, ambrisentan and macitentan

The combination of ambrisentan and tadalafil was approved by the FDA in October 2015 as up-front therapy for PAH to reduce the risk of worsening disease and improve exercise ability. [187] In a prospective multicenter open-label trial by Hassoun et al, this combination significantly improved hemodynamics, right ventricular structure and function, and functional status in treatment-naïve systemic sclerosis patients with PAH. [188]

One study reported that warfarin provided no significant benefit in either systemic sclerosis–associated or idiopathic pulmonary arterial hypertension. [189]

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