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Understanding Interstitial Lung Disease

A magnifying glass on a pair of lungs
Cedars-Sinai Interstitial Lung Disease Medical Director Tanzira Zaman, MD

Tanzira Zaman, MD

Interstitial lung disease (ILD) is a single label for a group of about 200 conditions, defined by inflammation or scarring in the lungs, that make it hard to breathe and can cause weakness and coughing. Since ILD is rare and complicated, patients often face a long and confusing road to get the right diagnosis.

And an accurate diagnosis is crucial—ILD ranges widely in severity, and some forms are completely reversible. Only when patients understand their exact condition can they get into treatment, start to feel better and possibly regain the ability to do things they love.

"Patients often get labeled incorrectly, or they hear 'pulmonary fibrosis,' do an internet search and read something that makes them think they only have three to five years to live, when that's not true for them," says Dr. Tanzira Zaman, who treats patients and oversees the Interstitial Lung Disease Program. "Here at our center, when we first see patients, we figure out exactly what's causing the inflammation. Often, patients have a great response after we adjust triggers such as medicines and environments, and their outcomes are really good."

Here, Dr. Zaman clarifies the different forms of ILD and how they are diagnosed and treated, and shares the one step all patients should take to improve their quality of life.


"Patients often get labeled incorrectly, or they hear 'pulmonary fibrosis,' do an internet search and read something that makes them think they only have three to five years to live, when that's not true for them"


Types and causes of ILD

One category of ILD is lung conditions with inflammation—but no scarring—that can be caused by environmental factors or medications. With these forms of the disease, the goal is to control or stop inflammation to prevent eventual scarring.

The other subset of ILD is pulmonary fibrosis, a condition marked by scarring in the lungs. There are two types of pulmonary fibrosis:

  • Idiopathic pulmonary fibrosis (IPF) has no known cause and most commonly impacts men in their 60s or 70s. The damage caused by IPF cannot be repaired, though medicines and therapies can help relieve symptoms, slow the disease and improve quality of life. The ILD Center offers clinical trials, and for some people, a lung transplant might be possible.
  • In other forms of pulmonary fibrosis, a cause can be identified, and the condition can be treated and its progression stopped. This type of pulmonary fibrosis can be caused by autoimmune conditions, some medications, or environmental exposures either eaten or breathed in.


Emphasis on diagnosis

Most people will develop ILD because of multiple risk factors—some can be defined and some cannot. Long-term use of certain antibiotics, such as those taken for urinary tract infections, may cause ILD. So can chemotherapy or immunotherapy. Exposure to mold or bird feathers or droppings can cause ILD, as can occupational hazards. Coal miners or people who work with metal can be susceptible.

Dr. Zaman encourages patients to seek a physician who will take the time to listen carefully. The first thing she does with any new patient is take a thorough history to help reveal the underlying cause of ILD, if possible.

"Only when we have a great diagnosis can we develop a confident treatment plan," she says. "We take a great deal of time to talk with patients to figure out all the clues. It takes some detective work."

Then, tests such as bloodwork and special high-resolution CT scans that provide very detailed images of the lungs can detect underlying medical conditions contributing to ILD. A lung biopsy may be necessary when the other tests are inconclusive.



Treatment

If the cause can be identified, Dr. Zaman helps patients remove themselves from the harmful environmental or medical situations. Physicians at the ILD Center can work with patients' other physicians to adjust medications.

Then, targeted medication and oxygen therapy help treat the conditions.

One powerful tool Dr. Zaman recommends for every patient is Cedars-Sinai's Pulmonary Rehabilitation Program, where experts provide personalized education and supervised exercise regimens. The six-to-nine-week program can't improve the insides of a patient's lungs, but instead focuses on other health aspects that impact shortness of breath. Exercises target heart and muscle function, which can improve breathing. Nutrition counseling and breathing techniques can help ease symptoms, too.


In Discoveries: Air Supply


"This is an intervention that really improves qualify of life in people who feel limited by lung disease," Dr. Zaman says. "Often, patients are skeptical it can help them, especially if they're already active or work out a lot. But most of the time, it helps them breathe better and do more when they complete the program."

Additionally, pulmonary rehabilitation can be a great place to meet other people navigating the same or a similar condition.

"Patients don't necessarily know anybody else with their condition, and peer support can be really empowering," she says. "That pool of unknowns that comes with the diagnosis can become a lot smaller."

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