Interstitial lung disease

By Medically reviewed by hellodoktor


What is interstitial lung disease?

Interstitial lung disease is a large group of disorders which is described as progressive scarring of the lung tissue between and supporting the air sacs. The scar associated with interstitial lung disease may cause progressive lung stiffness, eventually affecting your ability to breathe and get the oxygen process into your bloodstream.

How common is interstitial lung disease?

Interstitial lung disease affected 595,000 people globally in 2013. This resulted in 471,000 deaths.

However, it can be managed by reducing your risk factors. Please discuss with your doctor for further information.


What are the symptoms of interstitial lung disease?

The most common symptom of the interstitial lung disease is shortness of breath. Almost all people with interstitial lung disease will experience breathlessness over time.

Other symptoms of interstitial lung disease include:

  • Cough, which is usually dry and nonproductive.
  • Weight loss, most often in people with COP or BOOP.

In most forms of interstitial lung disease, the shortness of breath develops slowly (over-months). In interstitial pneumonias or acute interstitial pneumonitis, symptoms come on more quickly (in hours or days).

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consulting with your doctor. Everyone’s body acts differently.


What causes interstitial lung disease?

Interstitial lung disease may occur when an injury to your lungs triggers an abnormal healing response. Ordinarily, your body generates just the right amount of tissue to repair damage. However, in interstitial lung disease, the repair process does not follow the correct process and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into your bloodstream.

This disease can be triggered by many things which including auto-immune diseases, exposure to organic and inorganic agents in the home or workplace, medications, and some types of radiation. In some cases, the cause is unknown.

Occupational and environmental factors

Long-term exposure to a number of organic and inorganic materials and agents can damage your lungs. These include:

  • Asbestos fibers
  • Bird protein (live pets and feather-containing products)
  • Coal dust
  • Grain dust
  • Mold from indoor hot tubs, showers and prior water damage
  • Silica dust

Medications and radiation

Many drugs can damage your lungs, especially:

  • Chemotherapy/immunomodulating drugs, such as methotrexate and cyclophosphamide
  • Heart medications, such as amiodarone (Cordarone, Nexterone, Pacerone) and propranolol (Inderal, Inderide, Innopran)
  • Some antibiotics, such as nitrofurantoin (Macrobid, Macrodantin, others) and sulfasalazine (Azulfidine)

Some people who have radiation therapy for lung or breast cancer show signs of lung damage for months or sometimes years after the initial treatment. The severity of the damage depends on:

  • How much of the lung was exposed to radiation
  • The total amount of radiation administered
  • Whether chemotherapy was also used
  • The presence of underlying lung disease

Medical conditions

Lung damage can be associated with the following autoimmune diseases:

  • Dermatomyositis/polymyositis
  • Mixed-connective tissue disease
  • Pulmonary vasculitis (microscopic polyangiitis)
  • Rheumatoid arthritis
  • Sarcoidosis
  • Scleroderma
  • Sjogren’s syndrome
  • Systemic lupus erythematosus
  • Undifferentiated connective tissue disease

After extensive evaluation and testing, an explicit cause may not be found. Disorders without a known cause are grouped together under the label of idiopathic interstitial pneumonias, which are tissue-based classifications.

Risk factors

What increases my risk for interstitial lung disease?

Factors that may make you more susceptible to interstitial lung disease include:

  • Age

Interstitial lung disease is much more likely to affect adults, although infants and children are sometimes affected.

  • Exposure to occupational and environmental toxins

If you work in mining, farming or construction or any reason that are exposed to environmental agents, your risk of interstitial lung disease may be increased.

  • Family history

There is evidence that some forms of interstitial lung disease are heritable and your risk of developing it is increased if close family members have the disease.

  • Radiation and chemotherapy/immunomodulatory drugs

Having radiation treatments to your chest or using some chemotherapy or immunomodulatory drugs may increase your risk of interstitial lung disease.

  • Smoking

Some forms of interstitial lung disease are more likely to occur in people with a history of smoking, and active smoking may make the condition worse, especially if there is associated emphysema.

Diagnosis &treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.


How is interstitial lung disease diagnosed?

Imaging tests of the lungs are usually done to identify the problem.

  • Chest X-ray

A simple chest X-ray is the first test in the evaluation of most people with a breathing problem. Chest X-ray films in people with interstitial lung disease may show fine lines in the lungs.

  • Computed tomography (CT scan)

A CT scanner takes multiple X-rays of the chest and a computer creates detailed images of the lungs and surrounding structures. Interstitial lung disease can usually be seen on a CT scan.

  • High-resolution CT scan

If interstitial lung disease is suspected, using certain CT scanner settings can improve the images of the interstitium. This increases the CT scan’s ability to detect interstitial lung disease.

Pulmonary function testing: A person sits in a sealed plastic booth and breathes through a tube. People with interstitial lung disease may have a reduced total lung capacity. They may also have a decreased ability to transfer oxygen from their lungs into their blood.

Lung biopsy: obtaining lung tissue to check over under a microscope is the only way to determine which type of interstitial lung disease a person has. There are several ways to collect lung tissue, which is called a lung biopsy:

  • Bronchoscopy: An endoscope is advanced through the mouth or nose into the airways. Tiny tools on the endoscope can take a sample of lung tissue.
  • Video-assisted thoracoscopic surgery (VATS): Using tools inserted through small incisions, a surgeon can sample multiple areas of lung tissue.
  • Open lung biopsy (thoracotomy): In some cases, traditional surgery with a large incision in the chest is needed to obtain a lung biopsy.

How is interstitial lung disease treated?

According to the type of this disease as well as the causes, the treatments are determined.


Depending on the underlying cause of interstitial lung disease, treatments fall into two categories: anti-inflammatories or anti-fibrotics. Interstitial lung disease that has a known inflammatory or autoimmune process may benefit from initial anti-inflammatory or immunosuppressing medications. If there is a known exposure, avoiding the inciting agent is a first step to treatment. Specifically for idiopathic pulmonary fibrosis, there are two medications now available for slowing the scarring process. Your doctor may work with other doctors, such as a rheumatologist or cardiologist, to optimize your care.

Oxygen therapy

Using oxygen can’t stop lung damage, but it can:

  • Make breathing and exercise easier
  • Prevent or lessen complications from low blood oxygen levels
  • Reduce blood pressure in the right side of your heart
  • Improve your sleep and sense of well-being
  • You’re most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.


Lung transplantation may be an option of last resort for people with severe interstitial lung disease who haven’t benefited from other treatment options

Lifestyle changes &home remedie

What are some lifestyle changes or home remedies that can help me manage interstitial lung disease?

Staying as healthy as possible is essential to living with interstitial lung disease. For that reason, it’s important to:

  • Stop smoking

If you have lung disease, the best thing you can do for yourself is to stop smoking. Talking to your doctor about options for quitting, including smoking cessation programs, which use a variety of proven techniques to help people quit. Beside that secondhand smoking can also be harmful to your lungs, don’t allow other people to smoke around you.

  • Have good nutrition

People with lung disease may lose weight because it’s uncomfortable to eat and the extra energy need to takes to breathe maintain. These people need a nutritionally rich diet that contains adequate calories. A diet-expert can give you further guidelines for healthy eating.

  • Remain active

As much as you can tolerate, continue to exercise and remain active to avoid deconditioning.

  • Vaccination

Respiratory infections can worsen symptoms of interstitial lung disease. Make sure you receive the pneumonia vaccine and an annual flu shot.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.


Review Date: December 25, 2016 | Last Modified: September 12, 2019

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