Pulmonary hypertension (PH) on its own is burdensome to the patients it afflicts, but when PH is combined with other diseases, the conditions can be devastating. In the case of pulmonary fibrosis and emphysema alongside PH, patients are especially prone to significantly worse cardiac and pulmonary function.
A study presented at the Annual Congress of the European Respiratory Society, as reported by a MEDNET news article, evaluated 79 patients with PH associated with respiratory diseases, including chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), and combined pulmonary fibrosis and emphysema (CPFE). Cardiovascular magnetic resonance imaging, pulmonary function tests, and right heart catheterization revealed worse symptoms overall for PH-CPFE patients. These patients had significantly lower right ventricular ejection fraction than PH-COPD or PH-ILD patients, meaning their hearts could not pump out as much blood. There was a further association between right ventricular dysfunction and combined emphysema fibrosis scores.
In terms of survival, Kaplan Meier curves showed a worse outcome for PH-CPFE patients. As suggested by the news article’s commentary, identifying fibrosis and emphysema in PH patients could give insight on patients’ prognoses. This could prove valuable in determining appropriate treatment paths for patients.
For example, Dr. John Granton, Director of the Pulmonary Hypertension Program at Toronto General Hospital, asserts that, “Early referral for consideration of lung transplant should occur in suitable patients. At present the role of specific treatment for pulmonary vascular complications (apart from long-term oxygen therapy) is unclear.”
Yet on the other hand, awareness of simultaneous conditions may not immediately translate into better patient care. “There are currently no approved therapies for PH associated with ILD or COPD and certainly none with CPFE,” said Dr. John Swiston, Director of the Pulmonary Hypertension Program at Vancouver General Hospital. “These patient populations should receive optimal care for both their ILD and/or COPD. PH associated with these two diseases alone or in combination should be the focus of further analysis.”
Demonstrated by a clinical case study of a 63 year-old man, CPFE can be a difficult-to-treat disease. In this case study, the man was treated with a cohort of drugs including cyclophosphamide, N-acetylcysteine, and anticoagulants, but to no avail. His conditions progressively worsened and led to the need for supplemental oxygen therapy. Even still, his PH worsened over the next two years, until off-label ambrisentan therapy was initiated. After this point, his haemodynamic parameters improved, but he still suffered from dysponea, or shortness of breath.
Cases such as these, as well as the results of the study comparing PH-CPFE to other PH-associated lung diseases, clearly identify a need for research on treating combination diseases.