Therapeutic effect of pirfenidone in the sugen/hypoxia rat model of severe pulmonary hypertension

What is it about?

Pulmonary Hypertension (PH) is a frequently lethal condition without cure. The current therapies remain essentially palliative and do not reverse the progressive pulmonary vascular obstruction leading to right ventricular heart failure. Therefore, new therapies are urgently needed for this life-threatening disease. Herein, we provide definitive evidence that pirfenidone, already used in the treatment of idiopathic pulmonary fibrosis, has beneficial effects on the progression of disease in the well-established Sugen/Hypoxia rat model of severe PH. Our findings should encourage clinical investigations and especially the evaluation of the use of pirfenidone in conjunction with current PH therapies. Since PH is a common complication of idiopathic pulmonary fibrosis that significantly contributes to morbidity and mortality, our present findings encourage also the evaluation of pirfenidone on disease progression in PH patients with pulmonary fibrosis. This notion is particularly relevant in the light of the fact that nintedanib, the second drug approved for the treatment of idiopathic pulmonary fibrosis, has been recently reported to have disadvantageous effects in experimental and human PH.

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