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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

Interstitial Lung Disease vs. Idiopathic Pulmonary Fibrosis

It is critically important to distinguish IPF from other types of lung disease.  There is a family of lung diseases that are termed “interstitial lung diseases” or ILD.  This collection of diseases has different causes and treatments.  Some are related to connective tissue diseases such as Lupus, while others may be related to viral infections.

Some doctors are “lumpers” and others are “splitters”.  Being a “splitter” means that for some doctors it is important to categorize and then further sub categorize each diagnosis.  Other doctors,  the “lumpers”, are more comfortable with large groupings- such as diseases that are treated with one type of therapy or another.  I am a “lumper” when it comes to ILD. The most important question is whether the lung process will respond to medicines that suppress the immune system (immunosuppresants such as prednisone).  In the world of ILD, most respond to prednisone whereas IPF dose not. Thus it is important to separate Idiopathic Pulmonary Fibrosis from all of the other interstitial lung diseases.

Can You Distinguish Idiopathic Pulmonary Fibrosis from Interstitial Lung Disease?

Idiopathic Pulmonary Fibrosis  lung tissueYou can distinguish the difference between Idiopathic Pulmonary Fibrosis and other interstitial lung diseases by examining lung tissue under a microscope.  The tissue of IPF patients has a very specific pattern.  This pattern is called UIP (usual interstitial pneumonia).  This term is a bit confusing because there is no infection despite having the word pneumonia in the phrase.  In some geographic areas UIP is used interchangeably with IPF.  Fibrotic foci can be seen during microscopic evaluation of IPF lung tissue.  These are small areas of active collagen deposition and are felt to be the engines of progressive fibrosis (scarring).  The lesions are unevenly distributed in the lung tissue, more prominently at the bases (bottoms) and in the periphery (outside) areas.  There is abnormal lung tissue adjacent to normal lung tissue.  Honeycomb change is also typically seen and is felt to be an advanced stage of fibrosis.

Featured Blog Posts

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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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