Interstitial Lung Disease (ILD)

Overview

Interstitial lung disease (ILD) is a group of disorders characterized by scarring and inflammation in the tissue surrounding the air sacs, blood vessels, and airways in the lungs. The scarring in the lungs can make the tissue stiff, which may make breathing difficult.

Interstitial lung disease can be caused by long-term exposure to hazardous materials or autoimmune diseases.

After the lung scarring occurs, interstitial lung disease is irreversible. While treatments can slow the progression of the disease, your breathing will not return to full function.

In severe cases, a lung transplant is a treatment option.

Interstitial lung disease causes

Interstitial lung disease can be caused by 200 different conditions. Causes of ILD include:

• Autoimmune diseases. You can develop ILD from having an autoimmune disease such as sarcoidosis, scleroderma, rheumatoid arthritis, or lupus.
• Breathing in harmful substances. You can develop ILD from breathing particles such as asbestos, tobacco smoke, or coal dust.
• Genetics. ILD can be passed down to children through genes. 

In many cases, the cause of ILD is unknown. Generally, this occurs in people over 60 years old.

Interstitial lung disease risk factors

Risk factors that increase your chance of developing interstitial lung disease include:

• Age. ILD is more common in adults.
• Exposure to toxins. Long-term exposure in the environment or through occupations such as farming, mining, or construction increases your risk of developing ILD.
• Gastroesophageal reflux disease (GERD). People with GERD are more likely to develop ILD.
• Smoking. People who smoke or have a history of smoking are more likely to develop ILD. 
• Radiation and chemotherapy treatments. Patients undergoing radiation or chemotherapy treatments are more likely to develop ILD or other lung conditions.

Interstitial lung disease symptoms

While symptoms vary from person to person, the most common symptoms associated with ILD include:

• Shortness of breath, most commonly with physical activity.
• Dry cough that does not produce phlegm.
• Extreme fatigue.
• Loss of appetite.
• Unexplained weight loss.
• Pain in the chest. 
• Trouble breathing. 
• Bleeding in the lungs.

Interstitial lung disease complications

Complications associated with interstitial lung disease include:

• Pulmonary hypertension. Pulmonary hypertension is high blood pressure in your lungs. When blood flow in the lungs becomes limited, the pressure rises in the pulmonary arteries. As the condition progresses, pulmonary hypertension can cause serious complications.
• Right-sided heart failure. If the heart’s right ventricle (lower right chamber) is forced to pump harder than usual to pump blood through the narrowed pulmonary arteries, you could experience right-sided heart failure. 
• Respiratory failure. During end-stage interstitial lung disease, respiratory failure may occur if low blood oxygen levels and increased pressure in the pulmonary arteries cause heart failure.

Interstitial lung disease diagnosis

Your doctor can diagnose ILD with a full medical history, physical exam, and diagnostic testing.

Diagnostic testing may include:

• Blood tests. A blood test can help diagnose autoimmune diseases associated with ILD.
• Spirometry. Spirometry is a lung function test that can help determine if you have problems getting air into or out of the lungs. 
• Pulse oximetry. A pulse oximetry test measures oxygen saturation in the blood.
• Chest X-ray. A chest X-ray is used to obtain images of your lungs and track the progression of your condition.
• Chest CT scan. A CT scan can provide more detailed images of the lungs to determine the severity of damage to the lungs or help determine the appropriate treatment options for your case.
• Bronchoscopy. A bronchoscopy is used to take a sample of lung tissue to confirm your diagnosis.
• Biopsy. A surgical biopsy may be necessary to collect a larger sample of lung tissue. 

Interstitial lung disease treatment

There is not a cure for interstitial lung disease. The goal of treatment for ILD is to slow the disease progression and enable you to breathe easier. 

Treatments for ILD include:

• Lifestyle modifications. Making lifestyle changes such as quitting smoking, eating a balanced diet, exercising, and staying updated on your vaccines will help you stay healthy while living with ILD.
• Avoidance. If you developed ILD from exposure to a toxic material or medication, avoid that substance.
• Medications. Your doctor will determine what medication is most appropriate for your case. 
• Anti-inflammatory medications such as prednisone can reduce swelling in the lungs. 
• Immune-suppressing medications can stop your immune system from attacking itself and damaging the lungs. 
• Antibiotic medications can stop further lung scarring.
• Oxygen therapy. Supplemental oxygen may be used to help increase oxygen levels in the body, which allows you to breathe easier.
• Pulmonary rehabilitation. Your doctor may recommend pulmonary rehab to help you maintain or improve your activity level.
• Lung transplant. Lung transplant is the last resort option for severe ILD. A lung transplant may be an option for patients younger than 65 who do not have other major health conditions such as cancer, heart, kidney, or liver failure.

When to Seek Care

Schedule an appointment with your doctor if you experience any breathing issues. Various conditions can cause lung damage, so early intervention and diagnosis are essential.