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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

A New Paradigm in the Treatment of Pulmonary Fibrosis

November 13, 2019 By Dr. Jeremy Feldman

The standard approach to treating pulmonary fibrosis involves first determining if the pattern is typical of IPF or if the pattern is not typical.  This assessment is primarily based on high resolution CT characteristics in combination with patient characteristics (age greater than 50, cough and shortness of breath, crackles and clubbing on lung exam). Patients whose CT scans were not typical of IPF then often proceeded to surgical lung biopsy.  

A New Study in New England Journal of Medicine

IPF lung tissue

A recently published study in the New England Journal of Medicine now challenges this approach.  Patients with pulmonary fibrosis were included.  60% of patients had a CT pattern typical of IPF and 40% of patients had a CT pattern that was not typical of IPF.  Patients had to have progressive lung disease as measured by worsening pulmonary function tests.  These patients were then randomized to either OFEV (nintedanib) or placebo for 52 weeks. Patients who received OFEV had substantially slower progression of their lung disease compared to the placebo group.  Importantly, patients were excluded from this study if they were taking azathioprine, mycophenolate, rituximab, cyclophosphamide, or prednisone at doses greater than 20mg per day.  

OFEV Treatment in Idiopathic Pulmonary Fibrosis 

The results of this study now challenge the standard approach to pulmonary fibrosis.  Whereas previously we have tried hard to separate IPF from non-IPF, we may no longer need to do that.  OFEV appears to be equally effective in patients with imaging that is not typical for IPF.  It may be that many of the patients with imaging that was not diagnostic of IPF actually had IPF.  In clinical practice, it is common to have a patient with imaging not diagnostic for IPF who proceeds to lung biopsy and the pathologic diagnosis is IPF.  

What’s Next?

This new study provides support for a new approach to patients with pulmonary fibrosis.  Perhaps the new approach will involve asking the following questions:  Does the CT scan look like definite or possible IPF?  Or does the CT scan look like another specific pattern (sarcoidosis, hypersensitivity pneumonitis, recurrent aspiration pneumonia)?  If the CT scan is consistent with definite or possible IPF then no lung biopsy is needed.  Proceed with treating the lung disease with OFEV.  In patients with lung disease that does not fit into the possible or definite IPF category, the next question is whether medicines that suppress the immune system may be beneficial.  

Combined with the recent study showing OFEV is beneficial in scleroderma associated pulmonary fibrosis, we are now armed with a new approach in many of our patients.  I suspect that over the next few years we will learn that OFEV is beneficial in not just scleroderma associated pulmonary fibrosis but also pulmonary fibrosis associated with other connective tissue diseases such as rheumatoid arthritis, dermato/polymyositis, mixed connective tissue disease and even some patients with lupus associated pulmonary fibrosis.  We now have reason to consider OFEV in a broad group of patients not just IPF and scleroderma.  Patients with fibrotic hypersensitivity pneumonitis and fibrotic NSIP (non-specific interstitial lung disease) are also likely to benefit based on the new data.

Many important questions remain.  In patients with autoimmune lung disease, when should we use medicines to suppress the immune system and when should we use antifibrotic therapy with OFEV?  Perhaps many of these patients will benefit from both therapies.  Stay tuned….

Filed Under: IPF Treatment

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IPF patient with doctor

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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