Pulmonary Fibrosis and Interstitial Lung Disease

Interstitial lung disease (ILD) is a large group of disorders that can affect the small air sacs (alveoli) or surrounding structures (interstitium) of the lung. Scarring in the lungs is referred to as pulmonary fibrosis (PF) or Interstitial Pneumonia(IP). The diagnoses of ILD and PF are sometimes used interchangeably. Idiopathic pulmonary fibrosis (IPF) is a type of scarring lung disease without a known cause.

Symptoms of ILD can include feeling out of breath and coughing. These are due to the inflammation and scarring that can block the normal function of the lung. The scarring in the lungs is not typically reversible.

At Henry Ford Health, we put time and effort into identifying the right diagnosis for your specific condition. We look for an underlying cause so we can make changes to treat inflammation or slow the progression of scarring. We also work to identify specific treatments that can improve your life with ILD.

Henry Ford’s expertise in ILD

Henry Ford’s comprehensive ILD program includes one of Michigan’s largest interventional pulmonology programs. Our team has the expertise to provide minimally invasive diagnosis and treatment. Medications, rehabilitation, clinical trials and lung transplant in select cases offer hope for our patients with ILD and PF. Here, you’ll find:

• Team care for ILD: Patients evaluated in the Henry Ford Interstitial Lung Disease Clinic undergo a thorough history and physical exam. In the ILD Multidisciplinary Conference, our pulmonologists (lung specialists) review each patient’s case with radiologists (experts in imaging) and pathologists (doctors who study tissue in the lab). Bringing multiple experts to your case means we consider a wide range of treatment options. We also work with our interventional pulmonologists and thoracic surgeons to offer bronchoscopy and surgical lung biopsy when necessary and safe. Meet our pulmonary team.
• Specialized ILD expertise: We specifically treat and understand ILD. That means we’re up to date on the latest treatments, including studies on new medication therapies for idiopathic pulmonary fibrosis.
• Pulmonary and Rheumatology Clinic: Some people who have ILD related to a rheumatologic condition can take advantage of our combined ILD-Rheumatology Clinic. You’ll see both types of doctors during one office visit. They meet with you together to provide a second opinion or help with a challenging phase. With both specialists in the room, we can answer specific questions for you and your primary pulmonologist or rheumatologist.
• Research and clinical trials: We engage in research to help develop and test new medications for people with ILD. Henry Ford also offers numerous clinical trials for aspects of health that can be related to ILD. Learn more about clinical trials.
• Transplant center: Henry Ford provides lung transplantation for some people with the most advanced ILD. Patients benefit from having all their care in one health system.

What is ILD and pulmonary fibrosis?

ILD refers to a category of lung disorders that involve the interstitium, the lacelike support structures of the lung. They also may involve the small air sacs of the lung (the alveoli). With ILD, these areas become inflamed, thickened and sometimes scarred. The scarring is called fibrosis.

The inflammation and scarring reduces the amount of normal lung available to bring in oxygen. That’s what causes ILD’s symptoms: shortness of breath and an unproductive (dry) cough.

The terms ILD, PF and interstitial pneumonia (IP) are often used interchangeably. ILD is an umbrella term for conditions that block your ability to breathe. Not everyone with ILD has scarring. ILD includes lung diseases such as pulmonary fibrosis, non-infectious pneumonia and vasculitis (blood vessel inflammation).

What causes ILD and pulmonary fibrosis?

Some kinds of ILD and PF don’t have any cause that doctors can identify. These types of PF are called idiopathic interstitial pneumonia (IIP). Idiopathic pulmonary fibrosis (IPF) — the most common type of IIP — is a progressive, scarring lung disease without a known cause.

Other types of ILD result from:

• Reaction to environmental exposures such as mold, woodworking dusts and living or working around birds
• Occupational exposures, such as asbestos or coal dust
• Reaction to medications or supplements
• Illnesses that affect other parts of the body, such as rheumatoid arthritis, lupus, scleroderma, myositis, vasculitis, inflammatory bowel disease and immunodeficiency

How is ILD or pulmonary fibrosis diagnosed?

Diagnosing ILD early is important, because lung scarring is not reversible.

Before your visit, we’ll go over your medical records. While you’re at Henry Ford, we ask detailed questions about your medical and family history, medications and supplements, home, hobbies and work. We’ll also perform a physical exam. This gives us clues to what you might have been exposed to and helps us identify the cause.

Several tests can help form our diagnosis, including:

• Pulmonary function tests: Our pulmonary function lab is on the same floor as our ILD clinic. You may be asked to perform simple tasks like blowing into a tube or walking for several minutes. These tests help us understand how well your lungs are working. We often repeat these tests if you have not had them in the past four weeks to look for any evidence of improvement or worsening. Testing for ILD usually takes about an hour. Learn more about pulmonary function tests.
• Computed tomography (CT): A specialized CT scan of the lungs can reveal the patterns of inflammation and fibrosis (scarring) that are trademarks of ILD.
• Blood tests: Blood tests can provide clues to systemic or autoimmune (when the body fights itself) disorders like lupus, myositis, scleroderma or rheumatoid arthritis.
• Lung biopsy: In some cases, a biopsy (a small tissue sample from your lung) can be helpful in diagnosing ILD. We examine the tissue under a microscope to confirm an ILD diagnosis. We may take a biopsy surgically or using minimally invasive (nonsurgical) techniques. We can also look inside your lungs using bronchoscopy, a thin tube (scope) that we insert through your nose or mouth. Learn more about diagnostic bronchoscopy and interventional pulmonology.

ILD and pulmonary fibrosis treatment at Henry Ford

Regardless of how serious your condition is, we search for options that can extend your life and help you improve your quality of life. We are not always able to identify the cause of ILD. In some cases, patients can recover even if we do not know how the disease began. 

How we treat ILD depends on the specific type of ILD. We may advise stopping a medication or avoiding a specific exposure in your environment that is causing the lung reaction. If you’re traveling from outside Detroit for treatment, we’ll coordinate with you and your primary pulmonologist to build a plan that works best for you.

 Henry Ford offers the full range of treatments for patients living with ILD, including: 

• Medication: We are up to date on the latest developments in medication therapy, including drugs that control inflammation and slow scarring progression. We’ll work with you to find a medication that helps you.
• Pulmonary rehabilitation: In our on-site fitness center, our respiratory therapists, pulmonary nurses and physical therapists offer customized, supervised exercise programs to help you breathe better. We coordinate your rehabilitation program with your other medical care for the best outcomes. Find out more about pulmonary rehabilitation.
• Referrals to other specialists: Some patients need care for other conditions that contribute to ILD or result from it. We work closely with specialists in sarcoidosis and pulmonary hypertension (PH) from our lung department at Henry Ford.
• Whole lung lavage for PAP: Whole lung lavage (WLL) washes protein build-up from the lungs to make breathing easier for people with PAP. We are one of only a few centers to provide WLL for both lungs in one procedure. Learn more about whole lung lavage.
• Lung transplantation: For ILD patients with advanced lung disease, a lung transplant may be the best option. Our successful lung transplant program means we offer the full range of treatment options from the same team of doctors you trust. Read more about lung transplant.