With vague symptoms, interstitial lung disease (ILD) is often overlooked or misdiagnosed. If that happens, the consequences can be grave.
- Interstitial lung disease is a family of afflictions that cause inflammation and scarring in the lungs.
- When enough scar tissue accumulates, the patient feels short of breath.
- For many patients, ILD is treatable.
- Yale Medicine's Interstitial Lung Disease Program patients may have the option to participate in clinical trials for new therapies.
Danielle Antin-Ozerkis, MD, and her colleagues are not just doctors—they are also detectives. As medical director of Yale Medicine’s Interstitial Lung Disease Program, Dr. Antin-Ozerkis specializes in treating patients with interstitial lung disease (ILD), a family of afflictions that cause inflammation and scarring in the lungs.
One disease, called idiopathic pulmonary fibrosis (IPF), can be especially hard to pin down. Its name literally means “lung scarring with no cause.” Early on, there may be no noticeable symptoms. Eventually, though, a person with this condition may feel short of breath because scar tissue has built up to the point where the lungs struggle to get enough oxygen to the bloodstream. People who experience this may not realize it is a serious symptom, says Dr. Antin-Ozerkis. “You might get winded and say, ‘Oh, I’m getting older.’ Or you have a cough, and it’s not bothering you that much. It’s insidious.”
In recent years new therapies have become available, and increased awareness has led more doctors to consider interstitial lung diseases as a potential cause for breathing troubles. A precise diagnosis is essential, says Dr. Antin-Ozerkis, because “there are different kinds of treatment depending on the type of disease. Even for IPF, we have medications that can slow the progression of disease. So the earlier we see patients, the better.”
Discovering the source
Identifying the source of a person’s breathing difficulty is the first step in making a diagnosis, says Dr. Antin-Ozerkis. There are three main categories of interstitial lung diseases, based on their cause: environmental, autoimmune and idiopathic (without cause).
The team listens to all complaints, searching for evidence that might explain the source of a person’s lung ailment. There are many things that can lead to interstitial lung disease, including certain medications, exposures as work and even other diseases, such as rheumatoid arthritis and scleroderma. “We’ll spend a long time with a patient to get a full history and then examine them carefully,” Dr. Antin-Ozerkis says.
Those clues can help doctors customize a treatment plan and provide valuable information about prognosis. Many interstitial lung diseases are treatable. And new drugs can help manage scarring of the lungs.Here’s what the detective work that results in diagnosis and management might look like for each category.
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When the cause is environmental
Exposure to environmental agents can cause lung disease. For some people, being around birds can trigger inflammation in the lungs. If a patient has pet parrots or works on a farm, those are important clues. Dust—organic and inorganic—and certain chemicals are other examples of environmental and occupational factors.
How environmental lung disease is managed: Get rid of the bird; avoid the dust. “If we remove the exposure early enough, we can reverse the inflammation,” Dr. Antin-Ozerkis says. There’s a big benefit to catching environmental factors early, before scar tissue has formed. “If patients come in with advanced scarring, we often can’t reverse it,” Dr. Antin-Ozerkis says. Maintaining lung function is the treatment goal. At Yale Medicine’s Interstitial Lung Disease Program, Associate Director Mridu Gulati, MD, a specialist in ILD and occupational medicine, focuses on solving this problem, with specialized expertise in helping patients deal with factors affecting their work and home environments.
When symptoms relate to autoimmune disease
How lung symptoms caused by autoimmune disease are managed: Knowing that a patient has connective tissue disease gives doctors immediately useful information about the cause of his or her pulmonary problems and access to effective treatments. “Once we know the reason, there may be specific medication that can suppress the inflammation and improve the breathing problems,” Dr. Antin-Ozerkis says.
When there is no known cause
People with pulmonary symptoms not caused by the environment or an autoimmune disease are typically diagnosed with idiopathic pulmonary fibrosis. Such was the case for a Connecticut resident who suffered from mysterious symptoms: shortness of breath, lightheadedness, chest pain and tingly fingertips. Doctors suspected that the root cause might be his heart, but tests showed that it was perfectly strong. At last, Erica Herzog, MD, PhD, director of Yale Medicine’s Interstitial Lung Disease Program, diagnosed him with IPF. This enabled doctors to take steps that helped him manage his condition.
Yale Medicine offers new IPF treatments: According to Dr. Antin-Ozerkis, new antifibrotic medications can slow development of scarring in the lungs. “New agents are coming to the forefront all the time,” she says, adding that “we are hoping to develop a cure.”
Patients at Yale Medicine’s Interstitial Lung Disease Program can enroll in clinical trials testing new treatments. Although some have scar tissue that is too advanced to help, others are eligible for experimental therapies. For instance, the patient described above had IPF that had advanced to the point where he might have been expected to live only a few years. But he was young and had a strong heart, making him eligible for a lung transplant. The operation was successful, and his prognosis has markedly improved.
‘A positive impact in all cases’
Interstitial lung diseases are not well known, and thankfully, IPF remains rare. That means symptoms can often be mistaken for those associated with other conditions. Doctors who treat ILD are intent on raising awareness so the disease can be caught early enough to reverse its effects.
But even when discovering the cause of a patient’s disease does not help with treatment, doctors at Yale Medicine’s Interstitial Lung Disease Program can ease symptoms.
“We can have a positive impact in all cases,” Dr. Antin-Ozerkis says. “We make sure we’re there for the patient in every stage. When the disease is diagnosed early, we want to be really aggressive about medication, trying to reverse as much lung disease as possible. When it comes to our attention later, we help with supportive care, which includes everything from supplemental oxygen and rehabilitation programs to support groups and lung transplantation.”