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Abstract
Pulmonary arterial hypertension (PAH) is a rare, but severe and life-threatening disease
characterized by vasoconstriction and remodeling of the pulmonary arterioles, leading
to progressive increase in pulmonary vascular resistance and ultimately to right-heart
failure. In the last two decades, significant progress in treatment of PAH has been
made, with currently 12 drugs approved for targeted therapy. Among these, the stable
prostacyclin analogues iloprost and treprostinil have been repurposed for inhalation.
The paper highlights the development of the two drugs emphasizing the rationale and
advantages of the inhalative approach. Despite substantial advances in the specific,
mainly vasodilatory PAH therapy, disease progression is mostly inevitable and mortality
remains unacceptably high. Thus, introduction of new drugs targeting the cancer-like
remodeling of the diseased pulmonary arteries is urgently needed. Inhalation offers
pulmonary selectivity and will hopefully pioneer the repurposing of novel highly potent
drugs for effective aerosol therapy of PAH.