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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

Is Pulmonary Fibrosis Hereditary?

Research for IPF

One of the most common questions my patients ask me is whether their children are at risk for developing Idiopathic Pulmonary Fibrosis – whether it’s hereditary. The vast majority of patients have sporadic IPF (no risk of passing the disease to their children).

There are a few genes that have been found to be associated with familial IPF. Evaluating families with multiple affected family members identified these abnormal genes. Based on the available data, most familial cases are inherited as autosomal dominant—this means that there is a 50% chance of inheriting the abnormal gene that may causes the disease. Only a single abnormal copy of the gene is enough to develop the disease.

Patients without a family history of IPF can have mutations in the same genes that are associated with familial disease. This occurs through somatic mutations—mutations that develop after conception. These types of mutations are not passed on to your children.

It is critical to emphasize that the genetics of IPF are poorly understood and scientists are just beginning to make real progress. Below are some of the candidate genes that have been identified.

  1. Surfactant Genes
    1. In health, these proteins are critical to normal lung function providing lubrication and allow normal lung inflation.
  2. Telomerase Genes
    1. In health, these genes coordinate the repair of damage to the genetic information of cells.
  3. Mucin Genes
    1. In health, mucin is produced by lung tissue and helps with lubrication and may be involved in cell-cell signaling and preventing infection

Who should worry about familial Idiopathic Pulmonary Fibrosis?

If you have IPF and have a first-degree relative with Idiopathic Pulmonary Fibrosis, then you are at dramatically increased risk for having familial disease that you could pass to your children.

If I have familial IPF do I have a different prognosis?

A large study of patients and family members with familial IPF compared their age of onset, pulmonary function tests, imaging studies and survival to non-familial IPF. No differences were found. This was still a small retrospective (looking backwards in time) study. As we gather more information about familial and non-familial IPF we will be better able to answer this question.

 

Featured Blog Posts

2015 Updated American Thoracic Society Guidelines for Idiopathic Pulmonary Fibrosis

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Easing Shortness of Breath in Pulmonary Fibrosis

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Tips for Dealing with IPF Pain

Recent Posts

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

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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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