Pulmonary Fibrosis - Medical Malpractice Lawyers

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Pulmonary Fibrosis - Medical Malpractice

Fibrosis means "scarring" and pulmonary fibrosis is a term for a disease that results in scarring of the lungs. It can be caused by many things, including sarcoidosis, infections, Wegener's granulomatosis, asbestosis, silicosis, radiation therapy and inflammatory conditions like rheumatoid arthritis and lupus. Certain medications can cause pulmonary fibrosis. Pulmonary fibrosis is a serious condition, with almost all patients dying within five years of diagnosis.

In hypersensitivity pneumonitis, fibrosis of the lung can become an immune reaction to inhaled occupational chemicals and inhaled organic dusts. The dust is usually contaminated by fungi, animal products and bacteria that contribute to the disease. Sometimes pulmonary fibrosis can be idiopathic and have no known cause. Some conditions called interstitial pneumonitis respond to immunosuppressive therapy.

The major symptoms of pulmonary fibrosis include poor exercise intolerance, coughing, and shortness of breath. The symptoms can vary over time and can sometimes show periods of relative remission. The cause of the disease is usually idiopathic and begins with a dry, unexplained cough. It progresses slowly and insidiously and exercise tolerance worsens. There is eventually shortness of breath even without exercise. In rare cases, the condition can be rapidly progressive with disability occurring over a few weeks or months. This is a condition called Hamman-Rich syndrome.

Pulmonary fibrosis is diagnosed by doing a careful history and physical examination. Crackles can occur in the chest which are generally dry. A chest x-ray or CT scan of the lungs may show fibrotic changes in the lungs or can appear relatively normal. The scarring of the lungs, when visible, is peripheral in nature. Small bubbles appear when alveoli come together to form larger bullae. These are often seen at the base of the lungs. Pulmonary function studies can be done which show a restrictive lung disease and effectively low lung volume. Air flow is diminished throughout the expiration and inspiration. The diffusing capacity of the lungs is also diminished.

The confirmation of the diagnosis is best done through a lung biopsy, which is generally an open lung biopsy or done through a thorascope. This places a tube into the lung cavity and takes biopsies of the peripheral parts of the lungs. A biopsy may be unnecessary if there are typical findings associated with pulmonary fibrosis.

There are limited options for idiopathic pulmonary fibrosis. The scarring cannot be reversed and can only be slowed down. The only real cure is having a lung transplant. There are some research trials ongoing on drugs that reduce the progression of the scarring.

Prednisone is used to slow down changes caused by the overactive immune system. The immune system plays a strong role in developing pulmonary fibrosis. Using corticosteroids like prednisone, lung inflammation and scarring is reduced. Some people respond better than others to this form of therapy. Lung specialists or pulmonologists must be involved in the care of patients with this disease.

Besides prednisone or other corticosteroids, immunosuppressants are used to treat pulmonary fibrosis. These include cyclophosphamide, azathioprine, methotrexate, cyclosporine, and penicillamine. In some patients, colchicine has been used and has been successful. Gamma interferon and related immunosuppressants have been tried with limited success. Oxygen is used to help pulmonary fibrosis patients get enough air.

Complications of pulmonary fibrosis include the development of blood clots like pulmonary emboli. The disease is progressive and this is why most patients are dead within five years. Other complications include heart failure, fatigue, swelling of the legs and overall fluid accumulation.

Idiopathic pulmonary fibrosis is worse in smokers so stopping smoking can slow the progression of the disease. There is a rare form of idiopathic pulmonary fibrosis which appears to be genetic in nature. This is a disease that is almost uniformly fatal in nature with the exception of those who receive a lung transplant, which can cure the disease.

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The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here