Interstitial Lung Disease

What are interstitial lung diseases?

Interstitial lung disease is the name for a group of 100 chronic lung disorders. These diseases inflame or scar the lungs. The inflammation and scarring make it hard to get enough oxygen. The scarring is called pulmonary fibrosis.

The symptoms and course of these diseases may vary from person to person. The common link between the many forms of the disease is that they all begin with an inflammation.

  • Bronchiolitis. This is inflammation of the small airways (bronchioles).
  • Alveolitis. This is inflammation of the air sacs where oxygen and carbon dioxide exchange in the blood takes places (alveoli).
  • Vasculitis. This is inflammation that involves the small blood vessels (capillaries).

The most common types of interstitial lung disease are: 

  • A drug-induced disease
  • Exposure to substances at work or in the environment 
  • Lung injury due to radiation

Fibrosis leads to permanent loss of your lung tissue’s ability to carry oxygen. The air sacs, as well as the lung tissue around the air sacs and the lung capillaries, are destroyed when the scar tissue forms.

The disease may run a gradual course or a rapid course. People that have it may notice variation in symptoms, from very mild to moderate to very severe. The condition may stay the same for a long time or it may change quickly. The course of the disease is unpredictable. If it progresses, the lung tissue thickens and becomes stiff. Breathing becomes more difficult.

What causes interstitial lung diseases?

The cause of interstitial lung disease is not known. Major contributing factors are smoking and inhaling environmental or occupational pollutants, such as inorganic or organic dusts.

Other contributing factors include:

  • Certain drugs or medicines
  • Certain connective tissue or collagen diseases and sarcoidosis
  • Family history
  • Radiation treatment

What are the symptoms of interstitial lung diseases?

The following are the most common symptoms for interstitial lung diseases. However, each person may experience symptoms differently. Symptoms may include:

  • Shortness of breath, especially with activity
  • Dry, hacking cough that does not produce phlegm
  • Extreme tiredness and weakness
  • Loss of appetite
  • Unexplained weight loss
  • Discomfort in the chest
  • Labored breathing, which may be fast and shallow
  • Bleeding in the lungs

The symptoms of interstitial lung diseases may look like other lung conditions or medical problems. Talk with your healthcare provider for a diagnosis.

How are interstitial lungs diseases diagnosed?

In addition to a complete medical history and physical exam, the healthcare provider may also request pulmonary function tests. These tests help to measure the lungs’ ability to move air into and out of the lungs. The tests are usually done with machines into which you breathe. They may include the following:

Spirometry

A spirometer is a device used to check lung function. Spirometry is one of the simplest, most common tests. It may be used to:

  • Determine how well the lungs receive, hold, and move air
  • Look for lung disease
  • See how well treatment is working
  • Determine the severity of a lung disease
  • Find out whether the lung disease is restrictive (decreased airflow) or obstructive (disruption of airflow)

Peak flow monitoring

This device is used to measure the how fast you can blow air out of the lungs. Disease-related changes can cause the large airways in the lungs to slowly narrow. This will slow the speed of air leaving the lungs. This measurement is very important in evaluating how well or how poorly the disease is being controlled.

Chest X-rays

This test takes pictures of internal tissues, bones, and organs.

Blood tests

Arterial blood gas may be done to check the amount of carbon dioxide and oxygen in the blood. Other blood tests may be used to look for possible infections.

CT scan

This test uses a combination of X-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. CT scans are more detailed than regular X-rays.

Bronchoscopy

This is direct exam of the main airways of the lungs (bronchi) using a flexible tube called a bronchoscope. Bronchoscopy helps to evaluate and diagnose lung problems, check blockages, take out samples of tissue or fluid, and help remove a foreign body. Bronchoscopy may include a biopsy or bronchoalveolar lavage.

Bronchoalveolar lavage

Removing cells from the lower respiratory tract to help identify inflammation and exclude certain causes.

Lung biopsy

Removing a small piece of tissue from the lung so it can be examined under a microscope.

How are interstitial lung diseases treated?

Because there are so many causes, treatment will vary. Some interstitial lung diseases do not have a cure. Treatment is aimed at preventing more lung scarring, managing symptoms, and helping you stay active and healthy. Treatment can’t fix lung scarring that has already occurred.

Treatments may include:

  • Lung transplant
  • Oral medicine, including corticosteroids to reduce inflammation and cyclophosphamide (Cytoxan) to suppress the immune system
  • Oxygen therapy, from portable containers
  • Pulmonary rehab

Check with your healthcare provider about getting flu and pneumococcal shots. Getting a flu shot every year can help prevent both the flu and pneumonia. In addition, pneumococcal bacteria can cause minor problems, such as ear infections, but can also develop into life-threatening illnesses of the lungs (pneumonia), the covering of the brain and spinal cord (meningitis), and the blood (bacteremia). Pneumococcal disease can be acquired by anyone, but children younger than age 2, adults ages 65 and older, people with certain medical problems, and smokers are at the highest risk.  

Key points about interstitial lung diseases

  • Interstitial lung disease is the name for a group of 100 lung disorders that inflame or scar the lungs.
  • The cause is not known. Major contributing factors are smoking and inhaling environmental or occupational pollutants.
  • The most common symptoms are shortness of breath, especially with activity, and a dry, hacking cough.
  • Tests that help measure the lungs’ ability to exchange oxygen and carbon dioxide are used to diagnose the condition. Blood tests and imaging tests may also be used to see how severe the problem is and monitor it over time.
  • The goal of treatment for people with the disease is to prevent more scarring and manage symptoms.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

  • Know the reason for your visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • Bring someone with you to help you ask questions and remember what your healthcare provider tells you.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your healthcare provider gives you.
  • Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
  • Ask if your condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if you do not take the medicine or have the test or procedure.
  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your healthcare provider if you have questions.

Barbara C. Cahill, M.D.

Patient Rating:

4.9

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Barbara Cahill, MD, is an associate professor in the Department of Medicine at the University of Utah School of Medicine and a Huntsman Cancer Institute investigator. She is certified by the American Board of Internal Medicine. Cahill specializes in pulmonary medicine and lung transplantation. Additionally, she has clincal interests in tuberculosi... Read More

Specialties:

Advanced Lung Disease, Critical Care, Lung Transplant, Pulmonary, Tuberculosis

Locations:

University Hospital
Lung Transplant, Clinic 3
(801) 585-3697
University Hospital
Pulmonary, Clinic 3
(801) 581-7806

Holly J. Carveth, M.D.

Patient Rating:

4.8

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Holly Carveth, MD, is an assistant professor in the Department of Medicine at the University of Utah School of Medicine and a Huntsman Cancer Institute investigator. She is certified by the American Board of Internal Medicine. Carveth specializes in pulmonary care. She completed her medical degree at the University of Hawaii, followed by residency... Read More

Specialties:

Cystic Fibrosis, General Pulmonary, Pulmonary

Locations:

University Hospital
Pulmonary, Clinic 3
(801) 581-7806

Estelle S. Harris, M.D.

Estelle S. Harris, MD is an Associate Professor of Medicine at the University of Utah Health Science Center. As a Pulmonary and Critical Care physician, her clinical interests include general pulmonary consultation, acute lung injury, non-invasive ventilation, neuromuscular abnormalities and diaphragm muscle dysfunction. Dr. Harris received her M... Read More

Specialties:

Critical Care, General Pulmonary, Pulmonary

Locations:

University Hospital
Pulmonary, Clinic 3
(801) 581-7806

John R. Hoidal, M.D.

Patient Rating:

4.4

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John Hoidal, MD, is a professor and chair of the Department of Medicine at the University of Utah and an investigator of the Huntsman Cancer Institute. He holds the Clarence M. and Ruth N. Birrer Presidential Endowed Chair. His research focuses on the genetic and molecular bases for nicotine addiction and the consequences of cigarette smoking. By ... Read More

Specialties:

General Pulmonary, Pulmonary, Pulmonary Fibrosis

Locations:

University Hospital
Pulmonary, Clinic 3
(801) 581-7806

Richard E. Kanner, M.D.

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4.6

4.6 out of 5

Dr. Kanner has retired from clinical practice and is no longer seeing patients outside the research setting.... Read More

Robert Paine III, M.D.

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5.0

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Robert Paine III, M.D. is an experienced Pulmonary and Critical Care Medicine physician who has been board certified in Internal Medicine, Pulmonary Medicine and Critical Care Medicine. He cares for outpatients with a wide variety of pulmonary problems and has particular interest in chronic obstructive pulmonary disease (COPD) and unexplained shor... Read More

Specialties:

Advanced Lung Disease, Critical Care, General Pulmonary, Pulmonary

Locations:

University Hospital
Pulmonary, Clinic 3
(801) 581-7806

Cheryl Pirozzi, M.D.

Patient Rating:

4.7

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Specialties:

Advanced Lung Disease, Pulmonary

Locations:

University Hospital (801) 581-2018

Sanjeev M. Raman, M.B.B.S., M.D.

Patient Rating:

5.0

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Sanjeev Raman, MD is an Assistant Professor of Medicine in the Pulmonary Division, University of Utah School of Medicine. He has been a member of the Pulmonary division faculty since 2009. He was recruited to the University of Utah to expand the Lung Transplant Program. Dr. Raman completed his fellowship training in Pulmonary and Critical Care Med... Read More

Specialties:

Advanced Lung Disease, Critical Care, Lung Transplant, Pulmonary

Locations:

University Hospital
Pulmonary, Clinic 3
(801) 581-7806
University Hospital
Lung Transplant, Clinic 3
(801) 585-3697

Chakravarthy B. Reddy, M.D.

Patient Rating:

4.8

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Chakravarthy Reddy, MD, is an Huntsman Cancer Institute investigator and assistant professor in the Department of Medicine at the University of Utah School of Medicine. He specializes in lung cancer, airway disorders, and malignant pleural effusion. He is interested in advancing non-surgical ways to diagnose and treat cancers that occur in the ches... Read More

Specialties:

Critical Care, General Pulmonary, Lung Cancer, Pulmonary

Locations:

University Hospital (801) 581-7806

Darin T. Ryujin, M.S., PA-C, M.P.A.S.

Patient Rating:

4.8

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Darin Ryujin, Assistant Professor (clinical) is a Physician Assistant at the Sleep Wake Center. He has provided patient care at the Sleep-Wake Center since 2003.  As a Sleep Medicine provider, his clinical interests include general and cross-cultural sleep medicine.Darin received his Master’s of Science from Marshall University and his Master’s of ... Read More

Wayne M. Samuelson, M.D.

Patient Rating:

4.9

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Wayne Samuelson, M.D. is a Professor of Medicine (Clinical) at the University of Utah School of Medicine. He received both B.A. (Sociology) and M.D. degrees from the University of Utah. He completed a residency in Internal Medicine and a fellowship in Pulmonary Disease at Duke University Medical Center. At the completion of his fellowship training ... Read More

Specialties:

Asthma, Critical Care, General Pulmonary, Pulmonary

Locations:

University Hospital
Pulmonary, Clinic 3
(801) 581-7806

Mary Beth Scholand, M.D.

Patient Rating:

4.9

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Dr. Mary Beth Scholand, MD is an assistant professor in Pulmonary Medicine. Her research and clinical interest is Interstitial Lung Disease. This interest encompasses Idiopathic Pulmonary Fibrosis, Autoimmune Lung Diseases and Sarcoidosis. She is the Director of the Interstitial Lung Disease Clinic. She runs clinical trials focused on a cure ... Read More

Specialties:

Advanced Lung Disease, Pulmonary, Pulmonary Fibrosis

Locations:

South Jordan Health Center (801) 213-4500
University Hospital
Pulmonary, Clinic 3
(801) 581-7806

Matthew K. Topham, M.D.

Dr. Topham has special expertise in pulmonary diseases and is board certified in pulmonary medicine. He sees inpatients at the University of Utah hospital and directs an NIH-funded laboratory at the Huntsman Cancer Institute. In the laboratory, he studies lung, colon, and prostate cancers hoping to identify new ways to treat these diseases by fin... Read More

Specialties:

General Pulmonary, Pulmonary

Locations:

A location has not yet been added by this physician.

University of Utah Hospital

50 North Medical Drive Rm 4325
Salt Lake City, UT 84132
Map

801-581-5943