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Diffuse interstitial pulmonary fibrosis
Alternative namesDiffuse parenchymal lung disease; Alveolitis; Idiopathic pulmonary fibrosis (IPF); Cryptogenic Fibrosing Alveolitis (CFA); Idiopathic pulmonary pneumonitis (IPP)
DefinitionA group of disorders characterized by inflammation and eventually scarring of the deep lung tissues, leading to shortness of breath .
Causes, incidence, and risk factors
Interstitial lung diseases are a group of diseases, caused by inflammation and scarring of the alveoli (air sacs) and their supporting structures (the interstitium). This leads to the loss of the functional alveolar units and a reduction of the transfer of oxygen from air to blood.
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.
The interstitial lung diseases can be broken down into two large groups: those that have no known cause (idiopathic ILD) and those with an identifiable cause. One recent classification of idiopathic ILD includes the subclasses called: idiopathic pulmonary fibrosis/ usual interstitial pneumonitis (IPF/UIP), non-specific interstitial pneumonitis (NSIP), and acute interstitial pneumonitis (AIP). Sarcoidosis is a relatively common disorder that often causes interstitial lung disease.
Many occupational toxins, such as silica (sand) dust, asbestos fibers, coal dust, and cotton dust can cause ILD. Occasionally, hobbies such as keeping birds 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. Some medications (examples include bleomycin, amiodarone, and methotrexate) may cause ILD.
The risk factors, genetic predispositions and incidence 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.
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.
Cigarette smoking increases the risk of developing ILD and can cause the disease to be more severe.
Signs and tests
The following tests may be ordered:
The treatment depends upon the underlying cause of the disease. Most often, anti-inflammatory drugs, such as corticosteroids or immunosuppressing drugs, are given. If there is no specific treatment for an underlying disorder, supportive therapy is indicated. Supplemental oxygen is prescribed for people with low blood oxygen levels. Pulmonary rehabilitation can help people combat the debilitation that accompanies advanced ILD.
Quitting cigarettes can slow the progression of existing ILD.
Lung transplantation may be indicated in some patients with advanced ILD.
The stress of the illness can often be helped by joining a support group where members share common experiences and problems. See lung disease - support group .
Chances for recovery or progression of the disease depend on the underlying cause.
Calling your health care provider
Call your health care provider if you have shortness of breath at rest that lasts for any duration.
Avoid exposure to agents known to cause lung disease .
Update Date: 5/16/2002Darrell N. Kotton, M.D., Division of Pulmonary and Critical Care, Boston University School of Medicine, Boston, MA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT