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Idiopathic pulmonary
fibrosis |
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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
the pulmonary fibrosis treated?
The treatment options for idiopathic pulmonary fibrosis are very
limited. There is no evidence that any medications can help this
condition, since scarring is permanent once it has developed. Lung
transplantation is the only therapeutic option available. At
times, this diagnosis can be difficult to make even with tissue
biopsy reviewed by pathologists with specific experience in this
field. Research trials using different drugs that may reduce
fibrous scarring are ongoing. Since some types of lung fibrosis
can respond to corticosteroids (such as Prednisone) and/or other
medications that suppress the body's immune system, these types of
drugs are sometimes prescribed in an attempt to decrease the
processes that lead to fibrosis.
The immune system is felt to play a central role in the
development of many forms of pulmonary fibrosis. The goal of
treatment with immune suppressive agents such as corticosteroids
is to decrease lung inflammation and subsequent scarring.
Responses to treatment are variable. Once scarring has developed,
it is permanent. Those whose conditions improve with immune
suppressive treatment probably do not have idiopathic pulmonary
fibrosis.
The toxicity and side effects of treatments can be serious.
Therefore, patients with pulmonary fibrosis should be followed by
a lung specialist experienced in this condition. The lung
specialist will determine the need for treatment, the duration of
treatment, and will monitor the response to therapy along with any
side effects. Only a minority of patients respond to
corticosteroids alone, so other immune-suppressing medications are
used in addition to corticosteroids. These include
gamma-interferon, cyclophosphamide, azathioprine, methotrexate,
penicillamine, and cyclosporine. The anti-inflammatory medication
colchicine has also been used with limited success. Ongoing trials
are underway using newer drugs such as gamma interferon,
mycophenolate mofetil (Cellcept), and pirfenidone.
Pulmonary fibrosis can cause decreased oxygen levels in the blood.
A decrease in blood oxygen level (hypoxia) can lead to elevated
pressure in the pulmonary artery (the vessel that carries blood
from the heart to the lungs to receive oxygen), a condition known
as pulmonary hypertension, which can in turn lead to failure of
the right ventricle of the heart. Therefore, patients with
pulmonary fibrosis are frequently treated with supplemental oxygen
to prevent pulmonary hypertension.
There is also evidence that patients suffering from pulmonary
fibrosis may be at increased risk for blood clots that travel to
the lung (pulmonary emboli), and therefore anticoagulation (blood
thinning) therapy may be indicated
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