Diffuse interstitial lung disease

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Clubbing	Coal workers pneumoconiosis, complicated	Coal workers pneumoconiosis - stage II	Coal workers pneumoconiosis - stage II #2
Respiratory system

Diffuse interstitial pulmonary fibrosis is the name given to a group of disorders characterized by inflammation and eventually scarring of the deep lung tissues, leading to shortness of breath.

Diffuse parenchymal lung disease; Alveolitis; Cryptogenic fibrosing alveolitis (CFA); Idiopathic pulmonary pneumonitis (IPP)

Interstitial lung diseases (ILD) are a group of diseases caused by inflammation and scarring of the alveoli (air sacs) and their supporting structures (the interstitium). This leads to reduced blood oxygen levels.

There are dozens of different causes of ILD, and the classification systems are often confusing. In fact, many experts disagree on the best classification system for the interstitial lung diseases.

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The interstitial lung diseases can be broken down into two large groups:

• Those that have no known cause (idiopathic ILD) 
• Those with an identifiable cause

One recent classification of idiopathic ILD includes the following subclasses:

• Idiopathic pulmonary fibrosis/ usual interstitial pneumonitis (IPF/UIP)
• Non-specific interstitial pneumonitis (NSIP)
• Acute interstitial pneumonitis (AIP)

Many occupational substances such as silica (sand) dust, asbestos, coal dust, and cotton dust can cause ILD. People who work in industries with heavy exposures to known causes of occupational lung disease (for example, coal miners, sand-blasters, ship workers) are usually screened routinely for lung disease.

Occasionally, bird keeping or bathing in hot tubs cause some forms of ILD.

Many connective tissue diseases (such as rheumatoid arthritis, scleroderma, and lupus) can cause interstitial lung disease. Sarcoidosis is a relatively common disorder that often causes interstitial lung disease. 

Some medications (examples include bleomycin, amiodarone, and methotrexate) may cause ILD.

The risk factors, genetic predispositions, and rate of ILD depend on the specific disease being considered. Sarcoidosis, for example, is relatively common in some ethnic groups (African Americans), whereas idiopathic pulmonary fibrosis is quite rare.

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

Review Date: 05/03/2006
Reviewed By: David A. Kaufman, M.D., Assistant Professor, Division of Pulmonary, Critical Care & Sleep Medicine, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.