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Published on: November 18, 2018
Pulmonary hypertension associated with chronic lung diseases
Manuela Funke1, Thomas Geiser1, Otto D Schoch2
1Department for Pulmonary Medicine, Inselspital, University Hospital Bern, University of Bern, Switzerland.
Pulmonary hypertension (PH) in chronic lung diseases like COPD and ILD is challenging to treat. Current treatments for pulmonary arterial hypertension (PAH) may be harmful, and evidence-based therapies for PH in ILD or COPD are lacking.
Area of Science:
- Pulmonary Medicine
- Cardiology
- Respiratory Diseases
Background:
- Pulmonary hypertension (PH) is classified into groups, with Group 3 encompassing PH associated with chronic lung diseases such as COPD and ILD.
- PH in COPD and ILD presents management challenges due to limited evidence from randomized controlled trials (RCTs) with specific pulmonary arterial hypertension (PAH) drugs.
Purpose of the Study:
- To review the current understanding and management challenges of PH associated with chronic lung diseases.
- To discuss the potential risks and limited efficacy of existing PAH treatments in this patient population.
- To highlight the unmet need for evidence-based treatments for PH in ILD and COPD.
Main Methods:
- Literature review of international guidelines and clinical trials.
- Analysis of pathophysiological mechanisms linking PAH and fibrotic lung disease.
- Evaluation of past and emerging therapeutic strategies for PH in chronic lung conditions.
Main Results:
- No long-term RCT data exists for PAH-specific drugs in PH associated with COPD.
- A recent RCT in PH associated with ILD was halted early due to safety concerns and potential increased mortality.
- Existing PAH drugs may worsen gas exchange by increasing ventilation-perfusion mismatch in patients with chronic lung disease.
Conclusions:
- Specific PAH treatments may be harmful in PH associated with COPD and ILD.
- Current management requires careful consideration of individual pathophysiological mechanisms.
- There is a critical need for developing evidence-based, targeted treatments for PH in patients with ILD or COPD.
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