Pulmonary Hypertension Associated With Chronic Obstructive Lung Disease and Idiopathic Pulmonary Fibrosis

Yochai Adir; Sergio Harari


Curr Opin Pulm Med. 2014;20(5):414-420. 

In This Article

Abstract and Introduction


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.


Pulmonary hypertension may complicate the course of idiopathic pulmonary fibrosis (IPF), chronic obstructive pulmonary disease (COPD) and combined pulmonary fibrosis and emphysema (CPFE), and is increasingly recognized as a negative prognostic factor affecting the clinical manifestations, morbidity and mortality. IPF, COPD and CPFE are classified within the WHO group 3 pulmonary hypertension.[1] The degree of pulmonary hypertension in most patients will be mild to moderate, but a subgroup of patients will have a severe pulmonary hypertension. In the last 5th World Symposium on pulmonary hypertension,[2] the following definition was suggested:

The focus of this review is to describe the prevalence, pathogenesis, clinical implication and therapeutic approach to patients with pulmonary hypertension associated with COPD and IPF, with emphasis on the recent findings.