Pulmonary Conditions & Diseases: Idiopathic Pulmonary Fibrosis

Pulmonary Fibrosis is a form of interstitial lung disease (ILD) that causes inflammation and eventual scarring (fibrosis) in place of normal healthy lung tissue. Idiopathic Pulmonary Fibrosis (IPF) is a type of ILD resulting from an unknown reason or cause. Over time, the scarring can progress, take the place of healthy lung tissue permanently, lead to low oxygen levels, and make it more difficult to breath.

What are the signs and symptoms?

• Persistent cough, often dry (nonproductive)
• Breathlessness / shortness of breath (dyspnea
• Tiredness or fatigue
• Rounded fingernail shape (clubbing)
• Loss of appetite
• Unexplained weight loss
• Your healthcare provider hears abnormal breath sounds when listing to your lungs

What are the risk factors and triggers?

Although much is known about the molecular mechanisms and risk factors that lead to IPF, it is not well understood why some people get it and others do not. The term “idiopathic” means unknown. There are common risk factors for IPF that include:

• Genetics (family members with an interstitial lung disease)
• Gastroesophageal reflux disease (GERD), also known as acid reflux
• History of or current cigarette smoking
• Male sex
• Age, most commonly over 50 years old
• Previously or currently taking certain prescription medications
• History of exposure to environmental substances (most commonly found in construction, farming or factory work)

How is it diagnosed?

Your healthcare provider will listen carefully to your symptoms, discuss your medical history and perform a physical exam. The physical exam will include listening to your lungs (specifically listening for crackling sounds while you breathe), as well as your heart, joints, fingernails and skin. The diagnosis of IPF can often be difficult as many other diseases, including other ILDs, can have similar symptoms. Other testing and imaging that may be required to make the diagnosis include:

• Pulmonary function tests: to measure how much air you can breathe in and out of your lungs
• 6 minute walk test: monitoring your oxygen levels while you are walking
• CT scan (computed tomography): a more detailed imaging picture of your lungs to identify if there is scarring present
• Sometimes a biopsy is required to make the diagnosis. This can be obtained by bronchoscopy (a procedure of inserting a small flexible tube into your mouth or nose down into your lungs to obtain a small sample of lung tissue), or by a surgical lung biopsy (a surgery involving small incisions in the side of your chest to remove small pieces of lung tissue).

What is the treatment?

There is not a cure for IPF. There are currently two medications approved for use in slowing down the progression of scarring. These medications are pirfenidone (Esbriet) and nintedanib (Ofev), but some people are not able to tolerate these medications due to the side effects. Treatment goals are to manage symptoms and prevent worsening of IPF. This can often be managed with use of supplemental oxygen, participation in a pulmonary rehabilitation program, preventive care, and treating other health conditions appropriately. In some cases, a lung transplant may be an option. There may be opportunities for patients to participate in clinic research trials of novel therapies.

The Advanced Lung Disease program at Piedmont Healthcare will take a comprehensive approach to diagnosing, treating and providing specialty care for patients with interstitial lung disease. We are committed to providing our patients with the highest quality evidence based care, as well as support and educational opportunities, to improve understanding of the disease state and quality of life.