Interstitial Lung Disease (Pulmonary Fibrosis)

What are interstitial lung diseases?

Interstitial lung disease, or ILD, is a common term that includes 130 to 200 chronic lung disorders, which may be:

• chronic
• nonmalignant (non-cancerous)
• noninfectious

Interstitial lung diseases are named after the tissue between the air sacs of the lungs called the interstitium – the tissue affected by fibrosis (scarring).

Interstitial lung diseases may also be called interstitial pulmonary fibrosis or pulmonary fibrosis.

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

• bronchiolitis – inflammation that involves the bronchioles (small airways)
• alveolitis – inflammation that involves the alveoli (air sacs)
• vasculitis – inflammation that involves the small blood vessels (capillaries)

Most interstitial lung diseases are diagnosed as pneumoconiosis, a drug-induced disease, or hypersensitivity pneumonitis. The other types are:

• sarcoidosis
• idiopathic pulmonary fibrosis
• bronchiolitis obliterans
• histiocytosis X
• chronic eosinophilic pneumonia
• collagen vascular disease
• granulomatous vasculitis
• Goodpasture’s syndrome
• pulmonary alveolar proteinosis

What causes interstitial lung diseases?

The cause of interstitial lung disease is not known, however, a major contributing factor is thought to be inhaling environmental pollutants. Other contributing factors include:

• sarcoidosis
• certain drugs or medications
• radiation
• connective tissue or collagen diseases
• family history

Treatment for interstitial lung diseases:

Specific treatment will be determined by your physician based on:

• your age, overall health, and medical history
• extent of the disease
• your tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference