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Pulmonary Hypertension with COPD or Pulmonary Fibrosis

What is Pulmonary Hypertension with COPD Idiopathic Pulmonary Fibrosis (IPF)?

Pulmonary hypertension (PH) can occur in people who have chronic lung diseases, such as COPD (chronic obstructive pulmonary disease) or idiopathic pulmonary fibrosis (IPF).

The cause of PH was long thought to be due to chronically low oxygen levels, but the exact cause is not known.

Because the nature of these diseases is complex, they can be hard to treat.


COPD and pulmonary fibrosis are both chronic lung diseases that make it difficult for a person to breathe. However, these two diseases cause different kinds of damage to the lungs and have different causes.

History of smoking causes most COPD cases. In COPD — also known as chronic bronchitis — the airways and sacs in the lungs become blocked making breathing difficult.

IPF is the build-up of scar tissue in the lungs making it difficult for patients to breath. In IPF, the cause is unknown.

Severe Pulmonary Hypertension with Chronic Lung Disease Treatment

Treating PH associated with chronic lung disease is challenging.

People with lung disease do not necessarily respond the same way as those without lung disease do.

It is important to weigh the risks and benefits of PH therapies in the setting of underlying lung disease.

Based on clinical experience and lack of evidence for great benefits in people with mild PH, the team at the UPMC Comprehensive Pulmonary Hypertension Program focuses on treating severe PH.

People with severe PH are at highest risk of death and are most likely to be helped.

Given the complex nature of this diagnosis, we often recommend that our patients:

In our program, we work closely with the Emphysema Clinical Research Center and Simmons Center for Interstitial Lung Disease, through which research studies often are available.

We are happy to evaluate patients for PH associated with chronic lung disease to explore available clinical trials and help form patient-specific treatment plans.

Our expertise in PH, COPD, pulmonary fibrosis, and lung transplantation allows our patients to explore all available treatment options for this complicated disease.

Pulmonary hypertension with COPD treatment

PH with COPD falls into three categories:

  • Mild
  • Moderate
  • Severe

Treatment options for PH with COPD depends on the severity of the disease.

Treatment for mild PH with COPD includes lifestyle changes, such as quitting smoking and exercising.

As PH with COPD worsens, the treatment options change.

Treatment for moderate or severe PH with COPD includes:

  • Medications
  • Oxygen therapy
  • Pulmonary rehab
  • Surgery

Pulmonary hypertension with idiopathic pulmonary fibrosis treatment

IPF causes permanent and irreversable lung scarring.

Treatments for PH with IPF work to slow the progression of the disease and improve the quality of life.

Treatment options include:

  • Medications
  • Oxygen therapy
  • Pulmonary rehab
  • Surgery

Learn More About COPD and Other Breathing Disorders

UPMC Patient Education Materials on Breathing Disorders (links open a new window):

Make an Appointment for Severe PH Today

To make an appointment at the UPMC Comprehensive Pulmonary Hypertension Program, contact us at: