Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is a lung disease that occurs when lung tissue becomes scarred and damaged. The scarring of the lung tissue causes stiffness, making breathing difficult. The word “idiopathic” means it has no known cause, but there are many causes of pulmonary fibrosis.

Some causes of pulmonary fibrosis include:

  • Long-term exposure to certain toxins
  • Radiation therapy
  • Some medicines
  • Certain medical conditions

How fast pulmonary fibrosis worsens over time and how severe the symptoms are vary from person to person. These symptoms include:

  • Shortness of breath
  • Dry cough
  • Extreme tiredness
  • Unintended weight loss
  • Aching muscles and joints
  • The widening and rounding of the tips of the fingers or toes, called clubbing.

Pulmonary fibrosis has been found in children and infants, but this is not common. Idiopathic pulmonary fibrosis is much more likely to affect middle-aged and older adults. Other types of pulmonary fibrosis caused by connective tissue disease, can occur in younger people.

Certain factors increase the risk of pulmonary fibrosis. They are:

  • Smoking
  • Certain types of work
  • Cancer treatments
  • Genetics

Pulmonary fibrosis can cause many complications including:

  • High blood pressure in the lungs
  • Right-sided heart failure
  • Respiratory failure
  • Lung cancer and other lung problems

Pulmonary fibrosis is diagnosed when your doctor or other healthcare professional reviews your medical and family history and performs a physical exam.

Some other ways pulmonary fibrosis can be diagnosed include:

  • Imaging tests
  • Lung function tests
  • Biopsy/Tissue sample collection
  • Blood tests

The scarring and thickening of the lungs from pulmonary fibrosis cannot be repaired. And no current treatment has prevented pulmonary fibrosis from getting worse over time.

Treatments for pulmonary fibrosis are focused on:

  • Medicines
  • Oxygen therapy
  • Pulmonary rehabilitation
  • Lung transplant

Being actively involved in your treatment and staying as healthy as possible are essential to living with pulmonary fibrosis. It is important to:

  • Stop smoking and avoid other irritants to the lungs
  • Eat well
  • Exercise regularly
  • Take time to rest
  • Get vaccinated
  • Follow your treatment plan

Pulmonary fibrosis is a lifelong condition that worsens over time. It can cause fear, depression, and stress.

Here are some tips to help you cope with your diagnosis:

  • Learn about your condition.
  • Spend time with family and friends.
  • Talk to your doctor or other healthcare professionals.
  • Join a support group.
  • Learn about palliative care services.

If you need medical attention for symptoms of pulmonary fibrosis or other lung diseases, schedule an appointment with a pulmonologist at our Ambulatory Care Center by calling (718) 670-5486.

All content of this newsletter is intended for general information purposes only and is not intended or implied to be a substitute for professional medical advice, diagnosis or treatment. Please consult a medical professional before adopting any of the suggestions on this page. You must never disregard professional medical advice or delay seeking medical treatment based upon any content of this newsletter. PROMPTLY CONSULT YOUR PHYSICIAN OR CALL 911 IF YOU BELIEVE YOU HAVE A MEDICAL EMERGENCY.