What is Interstitial Lung Disease? Don't Dismiss These Symptoms

With all the virus and flu bugs circulating, it’s easy to attribute shortness of breath, coughing, wheezing, and tightness in the chest to a routine illness, or even to your aging body. But very often, these symptoms are a sign of a serious problem with your lungs.

According to Michele Hart, RN, a pulmonary nurse clinician at Baystate Pulmonary Rehabilitation, one out of every 200 American adults over 70 has what’s known as an interstitial lung disease (ILD). Undetected and untreated, they can cause irreversible lung damage, limit your quality of life, and, in some cases, be fatal.

What is Interstitial Lung Disease?

“ILD actually refers to a group of over 200 conditions that cause inflammation and scarring—or fibrosis—in your lungs,” says Hart. “That scarring can lead to stiffness in the lung tissue which makes it difficult to inhale or exhale fully. ILDs can also damage the tissues between the small air sacs in your lungs and the blood vessels around them and reduce the lungs’ ability to absorb oxygen.”

That scar tissue, Hart says, causes people with ILDs to often experience shortness of breath, a dry cough, a tightness or discomfort in the chest, and a sense of feeling tired all the time.

She adds, “Of the 250,000 Americans who currently live with an ILD, 40,000 of them die each year because of the disease. ILDs and their symptoms are not something to be dismissed or taken lightly.”

What Causes Interstitial Lung Diseases?

Interstitial lung disease can be triggered by many different things. Some, says Hart, can be hereditary, especially pulmonary fibrosis (PF). “One hereditary form of PF affects 3-20% of all ILDs diagnoses. In fact, one in five people with PF have family history or ILDs.”

Beyond genetics, other causes of ILDs include:

• Autoimmune diseases, including Lupus, rheumatoid arthritis, sarcoidosis, and scleroderma
• Environmental and occupational exposures, such as mold spores, bacteria, asbestos, silica, coal dust, hard metal dust, and animal proteins, most notably those found in the droppings of caged or indoor birds
• Medications, including those used to treat cancer (chemotherapy drugs), abnormal heart rhythms (Amiodarone), inflammatory conditions (Methotrexate), urinary tract infections (Nitrofurantoin)
• Radiation treatments to the chest may also cause scarring of the lungs

How is Interstitial Lung Disease Diagnosed?

ILDs are diagnosed through a combination of physical exam, a series of tests, and imaging.

A physical exam typically begins with listening to a patient’s breathing. “When people with ILD breathe in and out, a crackling sound—similar to the sound Velcro being pulled apart—can be detected using a stethoscope,” explains Hart.

That’s often followed by pulmonary function tests that measure how well your lungs take air in and out. “These are simple, non-invasive tests that allow us to determine how much air your lungs can hold, how easy it is for oxygen to move in and out of your lungs, as well as your overall lung health,” says Hart.

Another test that offers insight is a six-minute walk test. “This test measures how far you can walk in a 6 minute period of time, on a flat surface. This measurement is referred to as functional capacity.”

In addition, a high-resolution CAT scan image of the lungs is often taken. This imaging provides a more complete picture of the type and/or extent of any ILD or lung damage.

Treating Interstitial Lung Disease

Treatment for ILDs varies depending on the type of ILD diagnosed and the severity of the disease.

Because the damage from ILDs is irreversible and progressive, treatment tends to focus on slowing the progression of the disease, relieving symptoms, and improving or maintaining quality of life. Options include:

• Oxygen therapy
• Pulmonary rehabilitation
• Medication
• Lung transplantation

Here’s a look at how each can help:

Oxygen therapy

Delivered through a mask or a tube below your nose, extra oxygen helps reduce breathlessness and fatigue and makes it possible for many people to maintain an active lifestyle. However, oxygen therapy is only given to individuals whose oxygen levels are 88% or lower.

Pulmonary rehabilitation

The aim of pulmonary rehabilitation is to improve daily functioning by increasing exercise capacity as well as supporting other aspects of daily living to positively impact overall quality of life. Pulmonary rehabilitation often includes physical exercise, breathing exercises, nutritional counseling, and emotional support to help with the management of anxiety, stress and depression.

Medication

The purpose of the ILD medication is to prevent the creation of additional scar tissue and to help with inflammation. Popular options include:

• Anti-fibrotics: Nintedanib and Pirfenidone
• Anti-inflammatories: Coricosteroids, Mycophenalate and Azathioprine

Lung transplantation

A treatment of last resort for the most severe cases of ILD, lung transplantation may involve one or both lungs. To qualify for a transplant, a patient must undergo extensive testing which will provide them with a lung allocation score (LAS). The LAS determines where an individual falls on the transplant wait list. As the disease progresses and the person’s LAS changes, so does their placement on the list. However, it’s important to note that as with any transplant surgery there are significant risks including infection, organ rejection and even serious side effects from the many medications required in advance of and after transplantation.

How Serious is Interstitial Lung Disease?

Interstitial lung diseases can be especially dangerous if undetected or untreated. They can cause irreversible lung damage, limit your quality of life, and, in some cases, be fatal. Because there are so many types of ILDs and the severity can vary from patient to patient, there is no one-size-fits-all approach to living with the various symptoms and treatments.

The best approach is to work with your healthcare provider to develop a plan that effectively manages your symptoms and works to prevent further damage. Your plan may include treating other health conditions that can contribute to ILD including acid reflux and anemia. Be sure to be honest with your provider about your lifestyle, past exposures that may have occurred, and any symptoms you’re experiencing. This will offer you the best chance for creating a plan that addresses your symptoms and provides the best quality of life for as long as possible.