Understanding Interstitial Lung Disease and IPF

Early signs of interstitial lung disease, including idiopathic pulmonary fibrosis (IPF), can often be mistaken for common ailments like a cough or acid reflux. Doctors caution that these seemingly ordinary symptoms may be indicative of a more serious underlying condition.

Meet the Expert

Nicole Ng, M.D., associate director of the Interstitial Lung Disease Program at Mount Sinai Health System, explains the importance of recognizing and addressing persistent symptoms.

What is IPF?

Defining Interstitial Lung Disease

IPF is one of over 200 lung conditions categorized as interstitial lung disease (ILD). These conditions affect the tissue surrounding the air sacs in the lungs, leading to inflammation and scarring. This scarring reduces lung elasticity and hinders oxygen transfer to the bloodstream.

While some ILDs are linked to environmental toxins or autoimmune diseases, IPF is diagnosed when a lung scan reveals characteristic scarring without a clear cause, according to Dr. Ng. Approximately 50,000 Americans receive an IPF diagnosis annually.

Who is at Risk for IPF?

IPF typically affects older individuals, and its gradual onset can lead to symptoms being dismissed as a natural part of aging. “You might notice that things you used to do easily… slowly become harder,” requiring more frequent breaks, explains Dr. Ng.

The nonspecific nature of early IPF symptoms often results in misdiagnosis or delayed diagnosis, sometimes for months or even years. Therefore, persistent symptoms warrant further medical investigation. IPF is most commonly diagnosed in people in their 60s and 70s, and is more prevalent in men than women.

Smoking is a known risk factor, and cessation is strongly recommended. A family history of IPF also increases risk.

Recognizing IPF Symptoms

  • Shortness of breath during exercise or daily activities
  • A dry cough that doesn’t go away
  • Severe or persistent fatigue
  • Unexplained weight loss
  • Clubbing (widening and rounding of fingertips or toes)

Treating IPF

While there is currently no cure for IPF, early treatment can help slow its progression, says Dr. Ng. Treatment options include:

Antifibrotics

These medications are specifically approved to slow the decline in lung function. Until recently, only nintedanib and pirfenidone were available. However, nerandomilast received FDA approval in October of last year, providing a third option for patients.

Pulmonary Rehab

This therapy focuses on exercise training, breathing techniques, and energy conservation strategies, potentially improving lung capacity and quality of life.

Oxygen Therapy

Supplemental oxygen may be required as the disease progresses, initially during activities that cause shortness of breath, and potentially on a more continuous basis in later stages.

Treating Associated Conditions

Conditions like reflux, sleep apnea, and pulmonary hypertension often occur alongside IPF and are addressed as part of a comprehensive treatment plan.

If you experience a persistent cough or changes in your breathing without a clear cause, it’s crucial to consult your doctor and follow up for further evaluation. For more information, visit beyondthescars.com.