Pulmonary hypertension in parenchymal lung diseases: any future for new therapies?

Pulmonary hypertension (PH) due to chronic lung diseases is associated with a poor prognosis, regardless of the underlying respiratory condition. Updated PH guidelines recommend optimal treatment of the underlying lung disease, including long-term oxygen therapy, in patients with chronic hypoxaemia despite the lack of randomized controlled clinical trials supporting this statement. So far, randomized controlled trials on drugs approved for pulmonary arterial hypertension (PAH) have yielded discouraging results in both interstitial lung diseases (ILD) and chronic obstructive pulmonary diseases (COPD) with PH. In some cases, the trials were terminated because of an increase in death and other major adverse events in the active treatment arm versus placebo. In cases of PH due to idiopathic pulmonary fibrosis (IPF), new investigative therapies use a combination of novel antifibrotic treatments and other treatments approved for PAH. The choice of robust end points as well as a target group of patients with specific haemodynamic criteria may help in the selection of innovative therapeutic strategies. The aim of this review is to discuss recent studies and clinical trials for the treatment of PH due to the main chronic respiratory diseases and discuss possible future scenarios for the evaluation of new therapeutic strategies.

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