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Idiopathic Pulmonary Fibrosis

Jeremy Feldman, MD

  • What is Idiopathic Pulmonary Fibrosis?
    • Idiopathic Pulmonary Fibrosis Definition
    • Symptoms of Idiopathic Pulmonary Fibrosis
    • Causes of Idiopathic Pulmonary Fibrosis
    • Who Gets Idiopathic Pulmonary Fibrosis
    • Gastro-Esophageal Reflux (GER) and Idiopathic Pulmonary Fibrosis
    • Interstitial Lung Disease vs. Idiopathic Pulmonary Fibrosis
    • Is Pulmonary Fibrosis Hereditary?
    • Research, Life Expectancy and Prognosis for Idiopathic Pulmonary Fibrosis
  • Diagnosing & Monitoring PF
    • Monitoring Progress of 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
  • PF 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
      • Update on Oxygen Use
    • Clinical Trials in Idiopathic Pulmonary Fibrosis
    • IPF & Leg Swelling
    • Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis
    • IPF Exacerbation
  • PF Advocacy & News
  • FAQ’s
    • Respiratory failure, NSIP, diet
    • Leg swelling, lung biopsy, side effects & pets
    • FAQ’s Enzyme Therapy, Progression of IPF & Oxygen
    • FAQ: Diarrhea, Fibrosis Reversal, IPF Symptoms
    • FAQ: Oxygen Flow, Anesthesia & Genetics
    • FAQ: Contagious Cough? Stem Cells, Familial IPF, Flying
    • FAQ: GERD, Fatigue & Environmental Exposure
    • FAQ: How to Travel to Your Doctor’s Office with Oxygen
    • FAQ: Lung Biopsy with Pulmonary Fibrosis
    • FAQ’s: Alcohol, Clinical Trials, Pulmonary Function Testing
    • FAQs: Emphysema, Exercise & Lung Biopsy

Exciting Research Developments in Pulmonary Fibrosis

June 27, 2016 By Dr. Jeremy Feldman

Research for IPFThe IPF community is happy to have 2 FDA approved medications.  However, our enthusiasm is tempered by the fact that neither Esbriet nor OFEV improve lung function. They both have been shown to slow the progression of IPF.  It is clear that more medications are needed to improve the outcomes of our patients.

Reata Pharmaceuticals, a relative new-comer to Pulmonary Fibrosis, has an exciting study ongoing looking at a novel molecule to treat IPF (the study is known as the LARIAT study).  The study medicine (Bardoxolone Methyl) acts to decrease inflammation and improve energy use inside cells.  The company recently modified their inclusion criteria in a way that will greatly increase the number of pulmonary fibrosis patients that may be able to participate.  The study medication is an easy to take once daily pill.  As with all phase 2 studies, patients are randomized to study drug or placebo (sugar pill).  In this study, after an initial 16 week period, the placebo group may switch to active study drug and patients already on study drug continue on study drug.  The primary endpoint of the study is six minute walk distance.  Eligible patients include those with IPF as well as other types of pulmonary fibrosis.

United Therapeutics, a company with a long track record in pulmonary hypertension therapeutics, is about to start a study of an inhaled medication in patients with pulmonary fibrosis who also have pulmonary hypertension (the study is known as RIN-PH-201).  These patients have been excluded from most previous studies and there is little high quality data to guide treatment decisions.  The study medication is an already approved inhaled therapy.

How do you find a study center?

The FDA maintains a website—clinicaltrials.gov that lists every clinical trial that is sanctioned by the agency.  The website is searchable by study name, disease type and study medication.  At the bottom of the page describing the study is a list of study sites with contact information.

Why participate in research for Pulmonary Fibrosis?

The current studies listed above allow patients to take approved IPF treatments so there is no risk of missing out on existing treatments.  Without patients who are willing to participate in clinical trials we would have no treatment options.  By participating in clinical research, patients are able to gain access to potentially effective medications years before they are commercially available.  Only through a commitment to research can we advance the care for all of our patients.  Learn more about participating in clinical research.

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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