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Idiopathic Pulmonary Fibrosis

Jeremy Feldman, MD

  • What is Idiopathic Pulmonary Fibrosis?
    • Idiopathic Pulmonary Fibrosis Definition
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    • Causes of Idiopathic Pulmonary Fibrosis
    • Who Gets Idiopathic Pulmonary Fibrosis
    • Gastro-Esophageal Reflux (GER) and Idiopathic Pulmonary Fibrosis
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    • Is Pulmonary Fibrosis Hereditary?
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    • CPET used in IPF
    • HRCT Scans Used to Evaluate IPF
    • Lung Biopsy To Diagnose IPF
    • Pulmonary Function Tests (PFTS)
    • Six Minute Walk Test To Monitor IPF
    • Stages of Idiopathic Pulmonary Fibrosis
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  • PF Treatment Options
    • Esbriet (pirfenidone) Used to Treat IPF
    • OFEV (nintedanib) Used to Treat IPF
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    • Oxygen Used to Treat IPF
      • Update on Oxygen Use
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Pulmonary Fibrosis and Pulmonary Hypertension – a New Treatment Option

April 22, 2020 By Dr. Jeremy Feldman

United Therapeutics announced positive results of their study looking at the role of Tyvaso in patients with pulmonary fibrosis and pulmonary hypertension.  This is a landmark event.  No study to date has shown positive results in this population. 

Patients with interstitial lung disease (broad category of lung disorders involving scarring of the lungs) of any etiology were included provided that they also had pulmonary hypertension by right heart catheterization.  They were then randomized to receive either Tyvaso (an already approved inhaled medication for PAH) or placebo.  The primary endpoint of the study was change in six-minute walk distance at the end of 16 weeks.  Patients who received active treatment with Tyvaso walked 21 meters further.  This difference was statistically significant and is also clinically meaningful.  Secondary endpoints were also improved.  

Until now, we did not have compelling trial data supporting our decision to treat patients with both pulmonary fibrosis and pulmonary hypertension.  This provides great reassurance to physicians who for the past 15 years were carefully treating patients with pulmonary fibrosis and PAH with medications approved to treat PAH.  

Patients who have both interstitial lung disease and pulmonary hypertension are often very symptomatic.  We hope that with this announcement we are entering a new era of treatment options for patients with these intersecting problems.  

Filed Under: IPF Treatment

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Idiopathic Pulmonary Fibrosis Treatment Options

What is IPF?

  • What is Idiopathic Pulmonary Fibrosis?
    • Symptoms of Idiopathic Pulmonary Fibrosis
    • Causes of Idiopathic Pulmonary Fibrosis
    • Who Gets Idiopathic Pulmonary Fibrosis
    • Gastro-Esophageal Reflux (GER) and IPF
    • Interstitial Lung Disease vs. Idiopathic Pulmonary Fibrosis
    • Is Pulmonary Fibrosis Hereditary?
    • Research, Life Expectancy & Prognosis for Pulmonary Fibrosis

Diagnosing IPF

  • Idiopathic Pulmonary Fibrosis Diagnosis
    • Chest X-rays Used in IPF
    • CPET used in IPF
    • HRCT Scans Used to Evaluate IPF
    • Lung Biopsy To Diagnose IPF
    • Pulmonary Function Tests (PFTS)
    • Six Minute Walk Test To Monitor IPF
    • Stages of Idiopathic Pulmonary Fibrosis
    • Complications of Idiopathic Pulmonary Fibrosis

Treating IPF

  • Pulmonary Fibrosis Treatment Options
    • Esbriet (pirfenidone) Used to Treat IPF
    • OFEV (nintedanib) Used to Treat IPF
    • Pulmonary Rehab for IPF Patients
    • Lung Transplantation for Pulmonary Fibrosis
    • Oxygen Used to Treat IPF
    • Clinical Trials in Idiopathic Pulmonary Fibrosis
    • IPF & Leg Swelling
    • Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis
    • IPF Exacerbation

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