Diffuse interstitial lung disease : Overview, Causes, & Risk Factors

Alternate Names : Diffuse parenchymal lung disease, Alveolitis, Cryptogenic fibrosing alveolitis (CFA), Idiopathic pulmonary pneumonitis (IPP)


Diffuse interstitial lung disease refers to a group of lung disorders in which the deep lung tissues become inflamed.

Overview, Causes, & Risk Factors

The lungs contain tiny air sacs (alveoli), which is where oxygen is absorbed. These air sacs open up or expand with each breath.

The tissue around these air sacs is called the interstitium. In people with interstitial lung disease, this tissue becomes stiff or scarred, and the air sacs are not able to expand as much. As a result, not as much oxygen can get into your lungs, and therefore to your body.

Interstitial lung diseases can be broken down into two large groups:

  • Those that have no known cause (idiopathic ILD)
  • Those with an identifiable cause or that occur along with other diseases

There are several types of idiopathic ILD. Idiopathic pulmonary fibrosis (IPF) is the most common type. Less common types include:

  • Acute interstitial pneumonitis (AIP)
  • Cryptogenic organizing pneumonia or bronchiolitis obliterans organizing pneumonia (BOOP)
  • Desquamative interstitial pneumonia (DIP)
  • Lymphocytic interstitial pneumonia (LIP)
  • Nonspecific interstitial pneumonitis (NSIP)
  • Respiratory bronchiolitis interstitial lung disease (RBILD)

There are dozens of different causes of ILD.

  • Autoimmune diseases (in which the immune system attacks the body) such as lupus, rheumatoid arthritis, sarcoidosis, and scleroderma
  • Certain infections
  • Certain medications (such as bleomycin, amiodarone, methotrexate, gold, infliximab, etanercept)
  • Radiation therapy to the chest to treat breast cancer, lymphoma, and other cancers
  • Working with or around asbestos, coal dust, cotton dust, and silica dust

Cigarette smoking may increase the risk of developing some forms of ILD and may cause the disease to be more severe.

Pictures & Images


Clubbing�may result�from chronic low blood-oxygen levels. This can be seen with cystic fibrosis, congenital cyanotic heart disease, and several other diseases. The tips of the fingers enlarge and the nails become extremely curved from front to back.

Coal workers pneumoconiosis – stage II

Coal workers pneumoconiosis - stage II

This chest x-ray shows stage II coal worker’s pneumoconiosis (CWP). There are diffuse, small light areas on both sides of the lungs. Other diseases�that may explain these x-ray findings include simple silicosis, disseminated tuberculosis, metastatic lung cancer, and other diffuse, infiltrative pulmonary diseases.

Coal workers pneumoconiosis, complicated

Coal workers pneumoconiosis, complicated

This picture shows complicated coal workers pneumoconiosis. There are diffuse, small, light areas (3 to 5 mm) in all areas on both sides of the lungs. There are large light areas which run together with poorly defined borders in the upper areas on both sides of the lungs. Diseases which may explain these X-ray findings include complicated coal workers pneumoconiosis (CWP), silico-tuberculosis, disseminated tuberculosis, metastatic lung cancer, and other diffuse infiltrative pulmonary diseases.

Respiratory system

Respiratory system

Air is breathed in through the nasal passageways, travels through the trachea and bronchi to the lungs.

Review Date : 1/22/2010
Reviewed By : Allen J. Blaivas, DO, Clinical Assistant Professor of Medicine, UMDNJ-NJMS, Attending Physician in the Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Veteran Affairs, VA New Jersey Health Care System, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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