Interstitial Lung Disease

Breathing is something most of us do without thinking. But for people with interstitial lung disease (ILD) (also known as pulmonary fibrosis or parenchymal lung disease), each breath can be a challenge. ILD refers to a large group of disorders that cause inflammation and scarring in the lungs—this lung scarring and lung damage particularly affect the lungs’ air sacs, making it harder to breathe and for oxygen to reach the bloodstream.

Although ILD can be a serious and life-changing condition, early diagnosis and the right treatment can help slow disease progression and improve quality of life. In some advanced cases, a lung transplant may be an option.

What’s Interstitial Lung Disease?

ILD is not just one disease. It’s a term used to describe more than 200 conditions that affect the interstitium, the thin tissue that surrounds the tiny air sacs in the lungs where oxygen passes into the blood.

When this tissue becomes inflamed or damaged, it thickens and stiffens, leading to scarring (fibrosis). Over time, this scarring makes it harder for the lungs to expand and contract, resulting in symptoms such as:

• Chronic cough
• Shortness of breath
• Decreased exercise tolerance
• Chest congestion
• Fatigue
• Unintended weight loss

Symptoms often appear gradually and worsen over time, sometimes going unnoticed until a significant reduction in lung function has already taken place.

What Causes ILD?

Some causes of ILD are known, while others aren’t. When the cause is unknown, it’s called idiopathic interstitial pneumonia. The most common type is idiopathic pulmonary fibrosis (IPF), a condition that often progresses to end-stage lung disease

Known causes of ILD include:

• Autoimmune diseases like rheumatoid arthritis, lupus, or scleroderma
• Certain medications, including some chemotherapy drugs
• Smoking and environmental irritants

Because ILD has many potential causes, getting an accurate diagnosis is an important first step in creating an effective treatment plan.

Diagnosing ILD

If your doctor suspects ILD, they’ll start with a review of your symptoms, medical history, and any possible exposures. Tests may include:

• Imaging, like chest X-rays or high-resolution CT scans
• Pulmonary function tests to measure lung function and capacity
• Blood work to look for inflammation or autoimmune markers
• Lung biopsy, if needed, to look at lung tissue under a microscope

In some cases, oxygen therapy may be recommended low oxygen levels are documented.

Understanding Idiopathic Pulmonary Fibrosis

IPF is a serious form of ILD with no known cause. It causes permanent scarring of the lungs, which worsens over time.

Common symptoms include:

• Shortness of breath during activity
• A dry, hacking cough
• Fatigue
• Chest discomfort

Treatment Options

ILD treatment depends on the type and severity of your condition. Common options include:

• Medications to reduce inflammation or suppress the immune system
• Antifibrotic therapy to slow lung scarring in pulmonary fibrosis
• Oxygen therapy to support easier breathing and oxygen levels
• Pulmonary rehabilitation to improve endurance and help with daily activities
• Lung transplant for advanced cases where other treatments are no longer effective

Can ILD Be Prevented?

You may not be able to prevent every case of ILD, but you can take steps to lower your risk:

• Avoid breathing in harmful substances like asbestos, silica, or chemical fumes
• Wear protective gear when working in hazardous environments
• Quit smoking and stay away from secondhand smoke
• Get regular health checkups, especially if you have an autoimmune condition
• Let your doctor know if you experience unexplained cough, breathing problems, or decreased exercise tolerance.

A healthy lifestyle—with good nutrition, movement, and stress management—can also help protect your lung health.

Moving Forward with Confidence

Interstitial lung disease can be complex, but you’re not alone. With early diagnosis, personalized treatment, and a team that’s committed to your care, it’s possible to manage symptoms, slow disease progression, and maintain a good quality of life.

If you’re living with ILD or have been referred for advanced care, the specialists at The Lung Center at Jackson Health System who focus on advanced lung disease are here to help.