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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

Pulmonary Fibrosis & Lung Cancer

November 23, 2015 By Dr. Jeremy Feldman

Diagnosing IPF

Unfortunately, having idiopathic pulmonary fibrosis does not prevent you from developing yet another serious lung problem.  Data from several studies have shown that patients with IPF are at higher (5-7 times greater) risk for lung cancer.  This risk is independent of smoking exposure.  We don’t have a clear explanation for this observation but it does inform our care of IPF patients.

Lung Nodule Observed in Chest CT Scan

A common situation is that we identify a nodule on the chest CT scan.  This creates anxiety for you and your doctor.  There is no one best strategy to further evaluate these types of nodules.  My approach is to start by determining how severe the IPF is.  In patients with very severe IPF who are not candidates for lung transplantation, I tend to move slowly. This is because the overall prognosis is not affected by the presence of an early lung cancer and patients with advanced IPF generally do not tolerate radiation therapy or surgery.

In patients with early IPF, I proceed in the same fashion as though they did not have IPF.  In patients with moderately advanced IPF and a lung nodule who are potential candidates for lung transplantation, I generally obtain a PET scan to help risk stratify the nodule.  A “hot” nodule (one that takes up radioactivity on PET scanning is more worrisome and I would usually proceed with needle biopsy.  A “cold” nodule (one that does not take up radioactivity on PET scanning) is much less likely to be cancer and I would usually follow this with periodic CT scans.

Lung Transplantation for IPF Patients with Suspected Lung Cancer

In patients with advanced disease that are candidates for lung transplantation, there is an imperative to determine if the nodule is an early lung cancer because the presence of cancer would prevent listing for lung transplantation.  If the nodule were easily biopsied with a needle I would proceed in that direction (generally CT guided).

The treatment of lung cancer in patients with IPF is a challenge.  Patients with early disease would be considered for surgical resection but the risks of progressive breathing problems after surgery are increased.  I am increasingly using a special type of radiation therapy to treat patients with significant pulmonary fibrosis who develop early lung cancer.  This preserves more lung tissue and limits the loss of lung function associated surgery.  Early results from research studies are encouraging.

Patients with IPF and advanced lung cancer are generally not treated with surgery.  Radiation therapy and chemotherapy are used.  If the IPF is advanced, watchful waiting and hospice are also appropriate strategies.

Filed Under: IPF Treatment

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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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