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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

Your IPF Questions Answered

March 10, 2016 By Dr. Jeremy Feldman

Pulmonary Fibrosis FAQWe appreciate our readers submitting questions.

Why Does Leg Swelling Develop in Patients with Idiopathic Pulmonary Fibrosis?

Early in IPF, common symptoms are cough and shortness of breath with vigorous exercise or walking up inclines.  As IPF progresses, there is progressive loss of lung tissue including blood vessels in the lungs.  Over time, this leads to increased work for the right side of your heart.

In response to having to work harder, the right side of the heart releases hormones that promote fluid retention. These hormones act on the kidneys to decrease the amount of salt and fluid that is removed from your body.  Most people have similar swelling in both legs but some patients may have one leg that is a bit more swollen than the other.  If there is a marked difference in the swelling in one leg compared to the other, we worry about a blood clot and may investigate further with an ultrasound exam of the more swollen leg.

Do I Need a Lung Biopsy to Diagnose IPF?

This is a very important question for patients with chronic fibrotic lung disease.  I have written about this in detail (see section on role of lung biopsy).  I will summarize a larger post on this topic.  IPF may be diagnosed with confidence if a patient has the right demographics (age > 50, absence of connective tissue disease) and a high resolution CT scan of the lungs that has the characteristic findings.

However, many patients have CT scans of their lungs that are not typical.  These patients should consider lung biopsy, especially if they have early disease.  Patients with advanced scarring or who are very ill with respiratory failure may not be able to undergo lung biopsy due to risks of the procedure.  For less ill patients, video assisted lung biopsy is a low risk procedure.

If I Don’t Tolerate one IPF Medicine, Does this Mean that I Won’t Tolerate the Other?

It is not uncommon for a patient to have side effects with either Esbriet (Pirefenidone) or OFEV (Nintedanib).  The good news is that the two drugs are completely unrelated and intolerance to one does not predict intolerance to the other.  For example, if a patient has severe diarrhea with Esbriet, this does not predict that they will have severe diarrhea with OFEV and vice versa.

Pets and Pulmonary Fibrosis

A reader asked if a dog allergy can lead to worsening of IPF.

There is no data that suggests that allergies to dogs can lead to pulmonary fibrosis or exacerbate IPF.  Enjoy your canine companions!

Filed Under: IPF Tips

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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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