What is pulmonary
fibrosis?
"Fibrosis" is a term used to refer to scarring, so pulmonary
fibrosis means scarring throughout the lungs. Pulmonary fibrosis
can be caused by many conditions including chronic inflammatory
processes (sarcoidosis, Wegener's granulomatosis ), infections,
environmental agents (asbestos, silica, exposure to certain
gases), exposure to ionizing radiation (such as radiation therapy
to treat tumors of the chest), chronic conditions (lupus,
rheumatoid arthritis), and certain medications.
In a condition known as hypersensitivity pneumonitis, fibrosis of
the lung can develop following a heightened immune reaction to
inhaled organic dusts or occupational chemicals. This condition
most often results from inhaling dust contaminated with bacterial,
fungal, or animal products.
In some people, chronic pulmonary inflammation and fibrosis
develop without an identifiable cause. Most of these people have a
condition called idiopathic pulmonary fibrosis (IPF) that does not
respond to medical therapy, while some of the other types of
fibrosis, such as nonspecific interstitial pneumonitis (NSIP), may
respond to immune suppressive therapy.
Synonyms (other names) for various types of pulmonary fibrosis
that have been used in the past include chronic interstitial
pneumonitis, Hamman-Rich Syndrome, and diffuse fibrosing
alveolitis.
How is
pulmonary fibrosis diagnosed?
Pulmonary fibrosis is suggested by a history of progressive
(worsening over time) shortness of breath with exertion.
Sometimes, during examination of the lungs with a stethoscope, the
doctor can hear crackling sounds in the chest. The chest x-ray may
or may not be abnormal, but a special x-ray test called a high
resolution CAT scan will frequently demonstrate abnormalities.
Lung function testing is distinctly abnormal.
The diagnosis can be confirmed by lung biopsy. An open surgical
biopsy, meaning that the chest wall must be surgically opened
under general anesthesia to remove a portion of lung tissue, may
be necessary to obtain enough tissue to make an accurate
diagnosis. The removed tissue is examined microscopically by a
pathologist to confirm the presence of fibrosis.
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