Pulmonary Fibrosis (cont.)
What are pulmonary fibrosis symptoms?
Symptoms of pulmonary fibrosis include shortness of
breath, coughing and diminished exercise tolerance. The severity of symptoms and the progression
(worsening) of symptoms over time can vary and are at least partially dependent
upon the cause of the fibrosis.
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.
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.
Last Editorial Review: 12/6/2006
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