Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is the most common type of interstitial lung disease and causes scarring inside the lungs, making breathing incredibly difficult as scar tissue forms. This progressive condition affects other surrounding structures such as connecting tissue and the alveoli (sacs of air within the lungs).

Causes of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is a specific type of pulmonary fibrosis for which the exact cause is unknown. The condition is more common in males and there are a few additional risk factors that may make an individual more likely to get IPF.

These risk factors include:

  • Being over the age of 50
  • Smoking
  • Having acid reflux disease
  • Working around dust/fumes that may be inhaled
  • Having a family member diagnosed with a similar lung disease

Signs & Symptoms of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis may be present in the body for an extended amount of time without showing any noticeable symptoms. When the scar tissue buildup continues to get worse, however, the following symptoms may occur:

  • A dry, hacking cough that does not go away
  • Difficulty breathing/shortness of breath
  • Chest pain or tightness
  • Swelling of the legs
  • Loss of appetite
  • Unexplained weight loss
  • Fatigue
  • Joint and muscle aches
  • Clubbing (when the tips of fingers and toes widen)

How Is Idiopathic Pulmonary Fibrosis Diagnosed?

Because idiopathic pulmonary fibrosis shares many of the same signs as other lung diseases, it can be difficult to diagnose without specialized care from a pulmonologist (doctor who treats lung problems). Your healthcare provider will conduct an exam with a stethoscope, ask about your symptoms and possibly order one or more tests in order to effectively diagnose this condition.

Tests may include:

  • Imaging scans such as a chest X-ray or CT scan
  • An exercise test or oxygen desaturation study to check blood oxygen levels
  • Pulmonary function tests (also called breathing tests)
  • Biopsy (surgery to remove a small amount of lung tissue)

Treating Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is a non-reversible condition that progresses over time, but seeking early diagnosis and treatment is critical to help the lungs perform better for longer. Once this condition is diagnosed, your provider may recommend one of the following treatments or a combination of them:

  • Medications to preserve lung function and slow down lung scarring
  • Oxygen therapy to help with breathing
  • Pulmonary rehabilitation, a special exercise program
  • Lung transplant

Tampa General Hospital’s lung and pulmonary experts treat adult patients who have severe lung conditions, including idiopathic pulmonary fibrosis. Patients are evaluated for transplant eligibility on a case-by-case basis using information and input gathered by TGH’s multidisciplinary team, as well as other factors. Both single- and double-lung transplants are performed at TGH.