DRUG INDEX
Pulmonary Fibrosis
What is pulmonary fibrosis?
Pulmonary fibrosis is scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions such as sarcoidosis , hypersensitivity pneumonitis , rheumatoid arthritis, lupus, asbestosis, certain medications, etc.
In some patients, chronic pulmonary inflammation and fibrosis develop without identifiable cause, a condition called idiopathic pulmonary fibrosis.
What are symptoms of pulmonary fibrosis?
Symptoms of pulmonary fibrosis include shortness of breath, coughing and diminished exercise tolerance.
How is pulmonary fibrosis diagnosed?
Pulmonary fibrosis is suggested by a history of progressive shortness of breath with exertion. Sometimes, during the lung examination the doctor can hear crackling sounds in the chest. The chest x-ray may or may not demonstrate abnormal lung tissues. Lung function testing is distinctly abnormal. A special x-ray test called a high resolution CAT scan can frequently demonstrate abnormalities. A lung biopsy will confirm the diagnosis.
What is the treatment of pulmonary fibrosis?
The treatment of idiopathic pulmonary fibrosis frequently involves corticosteroids (such as Prednisone) and/or other medications that suppress the body's immune system. The immune system is felt to play a central role in the development of most forms of pulmonary fibrosis. The goal of treatment is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Once scarring has developed, it is permanent.
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 to monitor the response and any side effects. Only a minority of patients respond to corticosteroids alone. Immune suppressing medications are used in addition to corticosteroids. These include cyclophosphamide, azathioprine, methotrexate, penicillamine, and cyclosporine. The anti-inflammatory medication, colchicine, has also been used with some success.
Pulmonary fibrosis can cause decreased oxygen in the blood. Decreased blood oxygen (hypoxia) can lead to elevated pressure in the pulmonary artery ( pulmonary hypertension ), which can in turn lead to failure of the right ventricle. Therefore, patients are frequently treated with supplemental oxygen to prevent pulmonary hypertension.
Research presented at the 1999 national meetings of the American College of Rheumatology suggested that there may be a subgroup of patients with pulmonary fibrosis that is deemed without an identifiable underlying illness who are found to have an unusual antibody in their blood called anti-neutrophil cytoplasmic antibody, or ANCA. This subgroup of patients may be especially responsive to immune suppressing medications.
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