What Is Interstitial Lung Disease?

lung health examination

 asiseeit/Getty Images

Interstitial lung disease (ILD) includes more than 200 conditions that cause inflammation and scarring of lung tissue called the interstitium. The damage done by interstitial lung disease makes it harder for oxygen to enter the bloodstream. In addition to the interstitium, other parts of the lungs may be affected, including the airways, the lining of the lungs, and blood vessels.

One Korean study reported in 2015 found the incidence of ILD is 70.1 per 100,000 people per year, which is much higher than what was previously thought. Here is what you need to know about interstitial lung disease, including types, causes, symptoms, diagnosis, and treatment.

Types of Interstitial Lung Disease

All the types of interstitial lung disease affect the interstitium, a system of tissues running through the lungs. The interstitium supports the alveoli, the tiny balloon-like sacs of the lungs. Blood vessels travel through the interstitium, allowing for the blood to receive oxygen and rid the body of any excess carbon dioxide. Most disorders of the interstitium thicken lung tissues with scarring, inflammation, and retention of fluid. That thickening eventually makes it difficult for the blood to absorb oxygen, causing symptoms of ILD.

Idiopathic pulmonary fibrosis is the most common type of interstitial lung disease, accounting for 20% of all the cases.

Some ILD disorders are:

  • Interstitial pneumonia: This is a lung infection affecting the interstitium.
  • Chronic silicosis: A lung disease related to occupation and caused by breathing too much silica dust.
  • Idiopathic pulmonary fibrosis: Chronic scarring of the interstitium of an unknown cause.
  • Nonspecific interstitial pneumonitis: This ILD disorder is caused by an autoimmune disease and leads to damage of the interstitium.
  • Connective tissue-related pulmonary fibrosis: Also caused by autoimmune diseases and connective tissue conditions, this type of ILD leads to inflammation and/or scarring of the lungs.
  • Hypersensitivity pneumonitis: This type of ILD is caused by inhalation of allergens or other harmful substances, such as mold.
  • Sarcoidosis: This inflammatory ILD condition of the interstitium causes swollen lymph nodes and swelling in the heart, eyes, joints, skin, and/or nerves.
  • Asbestosis: This ILD condition can be caused by exposure to asbestos and leads to scar tissue on the lungs and inflammation of the lungs.
  • Familial pulmonary fibrosis: This is a type of ILD that causes scar tissue buildup in the lungs. It is known for affecting two or more members of a family.
  • Desquamative interstitial pneumonitis: This ILD disorder causes lung inflammation and is more common in people who smoke.

Interstitial Lung Disease Symptoms

People with interstitial lung disease cannot get enough oxygen in their blood. As a result, they experience shortness of breath, especially with activity. As the condition worsens, ILD will affect the ability to breathe, even with rest.

Additional symptoms may include:

Symptoms of interstitial lung disease may get worse with time. You should see your doctor if you experience trouble breathing. Once a diagnosis is made, treatments can help manage inflammation and scarring.

Causes

The causes of interstitial lung disease fall into five broad categories. These are:

  • Exposure or occupation-related, such as asbestosis and hypersensitivity pneumonitis
  • Treatment-related ILD, such as from chemotherapy
  • Autoimmune diseases and other connective tissues diseases including rheumatoid arthritis or lupus
  • Genetics—some interstitial lung diseases are passed down among families
  • Idiopathic ILD is a grouping for those types that have no known cause.

Smoking is a major risk factor for interstitial lung disease. In fact, according to the American Lung Association, smoking is not only a risk factor for ILD, but can also make ILD worse.

Diagnosis

To make a diagnosis of interstitial lung disease, your doctor will start with a health history and physical examination. Your doctor will also request a variety of tests to measure pulmonary function. Testing may include:

Spirometry: This test uses a spirometer device to check lung function. Your doctor will want to see how well you breathe in and out and how easily and quickly you blow air of your lungs. This test is simple and can help your doctor assess how well your lungs are functioning, look for lung disease, determine the severity of the disease, and check for decreased or restricted airflow.

Peak flow monitoring: A peak flow monitor measures how fast a person can blow air out of their lungs. Lung diseases can cause lung airways to slowly narrow, making it harder to blow air out.

Imaging: A chest X-ray or computed tomography (CT) scan can help take a better look at the lungs. CT scans are more detailed than X-rays.

Bloodwork: An arterial blood draw may be done to look for amounts of carbon dioxide and oxygen in the blood. Other blood work, such as the metabolic profile, complete blood count (CBC), or antibody tests, can check for overall health and signs of infection.

Bronchoscopy: Using a flexible tube called a bronchoscope, a clinician directly exams the main airways of the lungs (the bronchi). A bronchoscopy can evaluate lung problems, look for blockages, treat any problems, and remove samples of tissue and fluid for further testing. The bronchoscopy may include bronchoalveolar lavage, a biopsy, or both.

Lung biopsy: This test takes a tissue sample from the lung to be checked under a microscope and look for signs of ILD conditions, including scarred tissue and inflammation.

Bronchoalveolar lavage: This test removes cells from the lower part of the respiratory tract to look for inflammation and rule out or determine causes. This test is often done if your doctor suspects bleeding in the lungs.

Treatment

Treatment for interstitial lung disease depends on the type and severity. Often, it focuses on relieving symptoms, slowing down disease progression, and improving a person’s quality of life.

Treatment for ILD may include:

Pulmonary rehabilitation: Most doctors recommend pulmonary rehabilitation for ILD to strengthen the lungs, stretch lung capacity, and help make breathing easier. One study reported in 2017 looked at the effectiveness of pulmonary rehabilitation for people with ILD. The researchers determined that pulmonary was effective for many people with ILD regardless of severity and cause. In fact, the therapy was beneficial for reducing exercise tolerance, managing symptoms, and improving quality of life. Pulmonary rehabilitation may include a variety of activities, including physical exercise, breathing techniques to improve lung function, emotional support, and nutritional counseling.

Supplemental oxygen: Oxygen therapy can be prescribed to improve breathing and the ability to be active. Supplemental oxygen can be delivered to match normal levels of oxygen saturation.

Anti-inflammatory drugs: Some anti-inflammatory drugs can damage the lungs, but there are ones that can help relieve ILD symptoms. Corticosteroid drugs, such as prednisone, are considered anti-inflammatory drugs and can be helpful for managing ILD. 

Immune-suppressing drugs: If an autoimmune disease is the source of ILD, your doctor may prescribe immune-suppressing medications to help with reducing damage to the lungs and progression of ILD.

Antifibrosis medicines: These medications are newer drugs that are believed to block pathways in the body that cause scar tissue to form. The two most commonly prescribed antifibrosis medications for ILD are Ofev (nintedanib) and Esbriet (pirfenidone), both of which continue to be studied to determine their effectiveness.

Lung transplant: A lung transplant is an option for people with progressive and advanced disease that has not responded to other treatments. You will need to be in good health and not have other serious conditions to qualify for a lung transplant.

Your doctor is in the position to determine the best treatment for you with your unique health situation.

Complications

Left untreated, interstitial lung disease can become severe and lead to life-threatening complications. This may include:

Respiratory failure: This is seen in late-stage, longstanding ILD. Respiratory failure is the result of your lungs are failing to pass oxygen to the bloodstream and remove carbon dioxide.

Pulmonary hypertension: Scar tissue, inflammation, and low oxygen levels can restrict blood flow and eventually result in high blood pressure in the arteries of the lungs.

Right ventricle heart failure (cor pulmonale): Interstitial lung disease can lead to changes in the structure and function of the right ventricle. These changes may cause the right ventricle to pump harder to get the blood moving through the lungs. Overexertion and strain can cause the ventricle to fail.

A Word From Verywell

The outlook for interstitial lung disease varies from person to person. It can be a progressive disease and damage caused by the condition cannot be reversed. Symptoms of the condition can be unpredictable and life-altering. Fortunately, treatment can be helpful in slowing down lung damage and managing breathing. In rare cases, a lung transplant may be needed. Your doctor is in the best position to diagnose ILD and recommend treatment options that may offer you the best outlook. 

Was this page helpful?
Article Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. Cleveland Clinic. Interstitial lung disease. Updated April 26, 2018.

  2. Choi W, Dauti S, Kim HJ, et al. Risk factors for interstitial lung disease: A 9-year nationwide population-based study. BMC Pulm Med. 2018;18, 96. doi:10.1186/s12890-018-0660-2

  3. Scallan J, Huxley VH, Korthuis RJ. Capillary fluid exchange: Regulation, functions, and pathology. Morgan & Claypool Life Sciences; 2010. Chapter 2, The Interstitium. 

  4. Sauleda J, Núñez B, Sala E, Soriano JB. Idiopathic pulmonary fibrosis: epidemiology, natural history, phenotypes. Med Sci (Basel). 2018;6(4):110. doi:10.3390/medsci6040110

  5. Meyer KC. Diagnosis and management of interstitial lung disease. Transl Respir Med. 2014;2:4. doi:10.1186/2213-0802-2-4

  6. American Lung Association. Interstitial lung disease (ILD). 

  7. Sanford Health Care. Categories and types of interstitial lung diseases.

  8. American Lung Association. What is spirometry and why it is done? Updated February 19, 2020.

  9. Tonelli R, Cocconcelli E, Lanini B, et al. Effectiveness of pulmonary rehabilitation in patients with interstitial lung disease of different etiology: a multicenter prospective study. BMC Pulm Med. 2017;17(1):130. doi:10.1186/s12890-017-0476-5

  10. Voiriot G, Philippot Q, Elabbadi A, et al. Risks related to the use of non-steroidal anti-inflammatory drugs in community-acquired pneumonia in adult and pediatric patients. J Clin Med. 2019;8(6):786. doi:10.3390/jcm8060786

  11. Collins BF, Raghu G. Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis. Eur Respir Rev. 2019;28(154):195022. doi:10.1183/16000617.5022-2019

  12. Panagiotou M, Church AC, Johnson MK, et al. Pulmonary vascular and cardiac impairment in interstitial lung disease. Eur Respir Rev. 2017;26(143):160053. doi:10.1183/16000617.0053-2016