Pulmonary hypertension associated with chronic obstructive lung disease and idiopathic pulmonary fibrosis

Curr Opin Pulm Med. 2014 Sep;20(5):414-20. doi: 10.1097/MCP.0000000000000084.


Purpose of review: Severe pulmonary hypertension worsens the prognosis of patients with chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF). With the aim of better understanding the pathogenesis of this event and identifying the possible targets for therapeutic intervention, a great deal of clinical and translational research is now focused on this relevant field of medicine.

Recent findings: Some studies that were published last year have helped to better define the clinical and physiological profiles of patients with COPD or IPF and severe pulmonary hypertension. The importance of pulmonary rehabilitation was confirmed, particularly in patients with pulmonary hypertension associated with IPF. Information on the use of drugs approved for the treatment of pulmonary arterial hypertension is still very limited, because of some limitations and selection biases in the studies' design. New strategies (i.e. the use of fasudil or sepiapterin in pulmonary hypertension associated with IPF) have been evaluated in animal models.

Summary: Pulmonary hypertension in COPD or IPF may range from mild to severe. When pulmonary hypertension is more advanced, it can drive a poor outcome. Therefore, future studies should focus on this subset.

Publication types

  • Review

MeSH terms

  • Animals
  • Humans
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy
  • Idiopathic Pulmonary Fibrosis / complications*
  • Idiopathic Pulmonary Fibrosis / physiopathology
  • Prevalence
  • Prognosis
  • Pulmonary Disease, Chronic Obstructive / complications*
  • Pulmonary Disease, Chronic Obstructive / physiopathology