Which medications in the drug class PAH, PDE-5 Inhibitors are used in the treatment of Scleroderma?

Updated: Nov 13, 2018
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Answer

PAH, PDE-5 Inhibitors

These agents may increase vasodilation in the pulmonary vascular bed.

Tadalafil (Adcirca)

Tadalafil is aphosphodiesterase type-5 inhibitor indicated for improving and increasing exercise capacity in patients with World Health Organization (WHO) class I pulmonary arterial hypertension. It increases cyclic guanosine monophosphate (cGMP), which is the final mediator in the nitric-oxide pathway. Tadalafil has been shown to improve systemic sclerosis associated pulmonary hypertension and Raynaud phenomenon. Tadalafil has an extended half-life and therefore can be administered once daily without compromising its effectiveness.

Sildenafil (Revatio)

Sildenafil promotes selective smooth muscle relaxation in lung vasculature, possibly by inhibiting phosphodiesterase type 5. This effect results in subsequent reduction of pulmonary artery pressure and increase in cardiac output.

Sildenafil has been shown to be effective in reducing pulmonary artery pressure in systemic sclerosis associated pulmonary hypertension. Because of its potent peripheral vasculature vasodilatory effects it has also been used for treatment of severe Raynaud phenomenon with digital ulcers. However, owing to the short duration of its vasodilatory effects, sildenafil has to be administered several times per day.


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