• Home
  • About Us

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

Non-Specific Interstitial Pneumonitis vs. Idiopathic Pulmonary Fibrosis

November 29, 2017 By Dr. Jeremy Feldman

HRCT showing fibrosis
High resolution CT chest: axial images through the middle of the lungs. Note the increased areas of white markings along the outer aspect of the lungs. These are areas of fibrosis.

Pulmonary fibrosis is a somewhat generic term that describes scarring in the lungs.  Idiopathic pulmonary fibrosis (IPF) is a very specific term that describes a specific disease process that leads to progressive scarring in the lungs.  During the evaluation of scarring in the lungs, your doctor considers a variety of diseases that can cause scarring in the lungs.  Two of the most common are IPF and non-specific interstitial pneumonitis (NSIP).  Both diseases cause cough and shortness of breath.  Both diseases lead to abnormal CT scans of the lungs.  However, there are some very important differences.

Symptoms of NSIP vs. IPF

Symptoms are not able to distinguish IPF from NSIP.  In some cases, cough may be more prominent in IPF but this is not reliable.  There is a broad overlap in ages for the two diseases.  In general, both affect patients over 50 years of age.  The physical exam may reveal prominent crackles in the lungs.  IPF patients are more likely to have clubbing of the fingers.

Testing

Pulmonary function testing is similar with the two diseases as well.  Even high resolution CT scans of the chest can have substantial overlap.  However the high resolution CT chest often provides enough information to separate the two diseases.  The classic pattern on CT scanning for NSIP is a diffuse haziness or “ground glass” that is found at the bases of the lungs.  There is an absence of honeycomb change (thickened scarred areas that look like a honeycomb).  In many patients the high resolution CT scan of the lungs provides enough information to confirm IPF or NSIP.

In some patients there is no way to distinguish IPF from NSIP without a lung biopsy.  At present, the best type of lung biopsy is a VATS (video-assisted thoracoscopic surgery).  This procedure involves using two ports or small holes in your chest and taking a couple of biopsies of the lung.  Bronchoscopic lung biopsy (biopsy of the lung through a small camera advanced down your throat and then directed into the lungs) is still not very reliable in making a confident diagnosis due to the small size of the biopsies obtained.  Click here to learn more about surgical lung biopsy in pulmonary fibrosis.

Why is it important to distinguish IPF from NSIP?

The treatments are entirely different.  For IPF we have compelling data that immunosuppression (medicines that suppress your immune system don’t work).  In fact a large study showed that patients treated with prednisone and azathioprine (a strong immunosuppressant) did worse than those not treated at all.  On the other, the treatment for NSIP is usually prednisone and other immunosuppressants.

Do all patients with NSIP respond to treatment with prednisone or other immunosuppressants?

No.  NSIP comes in two distinct types—cellular and fibrotic.   Cellular NSIP generally responds well to treatment.  Fibrotic NSIP is more difficult to treat.

Is there a role for IPF medications (OFEV and Esbriet) in the treatment of NSIP?

At the present time we have no data suggesting any benefit to using IPF treatments in NSIP.  However, studies are ongoing that are looking at this very question.  As of November 2017, I do not favor the “off label” use of these medications for several reasons:  1) the medications have substantial side effects, 2) the use of an unproven medication in place of a medication with a track record of efficacy is not a good plan, 3) the high cost of the medications makes getting insurance approval difficult when used in an indication other than IPF.

Filed Under: IPF Symptoms

Featured Blog Posts

wine with pulmonary fibrosis

FAQ’s: Alcohol, Clinical Trials, Pulmonary Function Testing

Edema & Leg Swelling

Pulmonary Fibrosis & Edema: Why Are My Legs Swelling?

sex with pulmonary fibrosis

Why Sex is Important for IPF Patients

Recent Posts

  • Interstitial Lung Disease and Progressive Pulmonary Fibrosis
  • Pamrevlumab Ineffective in Treating IPF
  • New Drugs Moving Forward for IPF

Search by Keyword (Examples: GERD, Coughing, Traveling, etc.)

Search by Category

Popular Articles

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

Disclaimer