Interstitial Lung Disease and Pulmonary Fibrosis

What Are Interstitial Lung Disease and Pulmonary Fibrosis?

Interstitial lung disease damages the tissue in and around the alveoli (air sacs). Pulmonary fibrosis is a type of interstitial lung disease that indicates a specific kind of damage.

“Not all forms of interstitial lung disease involve lung scarring,” says Dr. Kerri Aronson, pulmonologist at Weill Cornell Medicine’s Division of Pulmonary and Critical Care Medicine. “However, when the disease does include scarring, it’s known as pulmonary fibrosis.”

Types of Pulmonary Fibrosis

Doctors categorize pulmonary fibrosis by cause. Idiopathic pulmonary fibrosis, the most common type, doesn’t have a known cause. Other types include:

• Pulmonary fibrosis caused by autoimmune diseases, such as rheumatoid arthritis or lupus
• Pulmonary fibrosis from exposure to harmful materials, such as asbestos or silica
• Pulmonary fibrosis that runs in families

What Interstitial Lung Disease Feels Like

You may not experience symptoms at first. However, as the disease progresses, they’re more likely to occur. You may often be short of breath or feel extremely tired. Your chest may hurt.

Other symptoms of interstitial lung disease and pulmonary fibrosis may include:

• Dry cough
• Joint and muscle aches
• Low blood oxygen levels
• Rapid breathing
• Weight loss
• Widening of the fingers and toes (known as clubbing)

Many Diseases, Numerous Causes

Interstitial lung disease includes more than 200 conditions, according to the American Lung Association. With such diversity, it’s not surprising that these conditions can have a wide range of causes, including:

• Autoimmune diseases
• Chest radiation
• Exposure to animal dander or mold
• Inherited genetic changes
• Job-related exposure to hazardous dust, chemicals or fumes
• Lung infections, such as pneumonia
• Medications that affect the lungs
• Smoking

Treating Interstitial Lung Disease

Determining the cause of your disease helps guide treatment.

“We think about potential harmful exposures in the patient’s environment, so we can counsel how to remove them,” Dr. Aronson says. “We also consider how to treat other medical problems that might be associated with interstitial lung disease, like an autoimmune disease or gastric reflux.”

Factors such as cause, severity of symptoms, lung function and your overall health can help your doctor choose the most effective treatment for you. Options include:

• Medications to reduce lung inflammation, slow scar tissue formation or treat related conditions, like pulmonary hypertension
• Pulmonary rehabilitation to improve your lung function, exercise endurance and knowledge of how to live with lung disease
• Supplemental oxygen to reduce shortness of breath so you can be more active
• Lung transplantation (for severe disease that’s worsening) to improve life expectancy and quality of life

Over the long term, your pulmonologist will monitor you for disease progression, which may require adjustments to your treatment plan.

The Bottom Line

Interstitial lung disease presents a major challenge, but a pulmonologist can help you navigate it. Here’s what to remember:

1. Interstitial lung disease is a group of conditions (including pulmonary fibrosis, which causes lung scarring) that damages the tissue near and within the lungs’ air sacs.
2. You may experience symptoms as the disease worsens, including shortness of breath, fatigue and cough.
3. Many things can cause interstitial lung disease, including autoimmune diseases and certain medications, as well as environmental and job-related exposure to harmful materials.
4. Available treatments include medications, supplemental oxygen, pulmonary rehabilitation and, in severe cases, lung transplantation.

Looking to enjoy the best quality of life possible while living with lung disease? Find a doctor at Weill Cornell Medicine who can partner with you on long-term management.