What is interstitial lung disease?

What is interstitial lung disease?

Interstitial Lung Disease (ILD) is the name used to describe a large number of conditions that cause various degrees of inflammation and scarring in the lungs. The term interstitium refers to the affected tissue in the lungs that surrounds the air sacs (or alveoli) and the blood vessels that play important roles in oxygen and carbon dioxide exchange. The thickening and stiffness that result from inflammation and scarring affects the normal breathing pattern and exchange of oxygen and carbon dioxide that takes place when we breathe in and out. People with ILD have difficulty getting sufficient oxygen to meet the body’s needs particularly when they exert themselves. 

Among the many types of ILD are:

  • Idiopathic pulmonary fibrosis
  • Non-specific interstitial pneumonia
  • Hypersensitivity pneumonitis
  • ILD associated with connective tissue diseases
  • Occupational lung disease
  • Sarcoidosis

Symptoms

Symptoms

ILD symptoms include:

  • Increased difficulty breathing with daily activities or exercise
  • Shortness of breath at rest as disease progresses
  • Cough
  • Fatigue
  • Chest discomfort
  • Weight loss (in some patients)
  • Symptoms of other diseases causing ILD, such as joint pain and swelling or skin rash

Causes

Causes

Just as there are many different types of interstitial lung disease, there are many different causes. Among the most common are:

  • Long-term exposure to/inhalation of certain substances such as asbestos, silica, tobacco, and beryllium, either in the home or workplace
  • Diseases of the connective tissue such as lupus, scleroderma, and rheumatoid arthritis
  • Sarcoidosis, a disease that can cause small inflammatory nodules and scarring to form in the lungs and elsewhere in the body
  • Allergies to environmental substances including fungi and bacteria
  • Radiation treatment to the chest
  • Exposure to animal and bird droppings
  • Certain drugs that treat cancer, heart disease, and some antibiotics 

In some cases, there is no known cause for interstitial lung disease. Your doctor may refer to this as idiopathic pulmonary fibrosis or Idiopathic interstitial pneumonia.

Risk Factors

Risk Factors

Among the factors that raise an individual’s risk for ILD are:

  • Male gender
  • Smoking
  • Untreated gastroesophageal reflux disease
  • Exposure to toxic substances
  • History of COPD, tuberculosis, pneumonia and Hepatitis C
  • Older age (over 70)
  • Family history of ILD

Diagnosis

Diagnosis

Physicians diagnose ILD using a number of measures. In addition to conducting a physical examination and taking a family and work history, she or he may order a number of tests. These include:

  • Blood tests
  • Lung function tests, such as spirometry, which can help measure disease severity
  • An X-ray or CT scan to obtain images of your lungs
  • Bronchoscopy, an outpatient procedure that allows the physician to see inside the lungs, obtain small tissue biopsy and cultures
  • A surgical biopsy to remove small pieces of lung tissue for analysis 

ILD is usually a progressive disease, which means that the disease may result in worsening scarring in the lungs and symptoms over time. An early diagnosis and identifying the cause of ILD when possible are essential in slowing the course of the illness and keeping patients comfortable longer.

Treatment

Treatment

Treatment for ILD focuses on treating symptoms and addressing any underlying disease that is contributing to the condition. Physical therapy, supplemental oxygen, removal of exposures that may lead to ILD, and certain medications, such as antifibrotic drugs or immunosuppressants to reduce inflammation, can slow disease progression and help make patients more comfortable.

 

This content has been reviewed by the following medical editors.

Selim Arcasoy, MD

 

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