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Pulmonary Fibrosis
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Pulmonary fibrosis is a scarring disease of the lungs. It happens after repeated injury to the lungs, including the air sacs. The damage eventually causes scar tissue or fibrosis to occur within the lungs. Breathing becomes a problem a problem and you develop a dry cough. Current treatments for pulmonary fibrosis include using medications that improve the life of the sufferer and improve breathing.
Symptoms of pulmonary fibrosis include being chronically short of breath, having a dry cough, having an unexplained weight loss, having aching muscles or aching joints and being tired all the time. Breathing problems tend to get progressively worse so that your exercise tolerance worsens. Some people get sick very quickly and others have the symptoms come on very gradually.
In pulmonary fibrosis, damage to the alveoli causes scar tissue to develop in the interstitium or connective tissue of the lungs that separates the air sacs. The interstitial tissue becomes very stiff and the air sacs cannot expand to their full potential. The air sacs become stiff and dry so that air exchange is difficult. The healing process does not occur and the fibrosis persists.
Causes of lung fibrosis include occupational hazards and environmental factors, such as exposure to toxins, pollutants and other chemicals. Silica dust and asbestos can cause pulmonary fibrosis. Grain dust, bird or other animal droppings, sugar cane and other agricultural grains, if exposed to for long enough, can cause a person to develop pulmonary fibrosis. Radiation to the lung area can cause damage to the lungs up to several years after exposure.
Medications can damage the lungs, including chemotherapy drugs, propranolol, amiodarone and some psychiatric medications. Some antibiotics can yield pulmonary fibrosis. Chronic gastroesophageal reflux can send acid to the lung tissue and can cause pulmonary fibrosis to occur.
Having a history of tuberculosis or chronic pneumonia can lead to permanent lung scarring and fibrosis. Diseases like lupus, dermatomyositis, sarcoidosis, rheumatoid arthritis and Sjogren's syndrome are autoimmune diseases that can cause pulmonary fibrosis.
Sometimes the cause of the disease is unknown and the patient has idiopathic pulmonary fibrosis. No one knows what the exact cause of the fibrosis is but some doctors believe it has to do with tobacco abuse or viral illnesses. Genetics is considered to be part of getting the disease.
Those who are more likely to get pulmonary fibrosis are those who are middle aged or older, men, exposure to environmental or occupational toxins, having radiation or chemotherapy, having a history of smoking, having relatives with the disease, and being exposed to certain viruses.
The diagnosis of pulmonary fibrosis can be difficult. It can mimic other diseases that have to be ruled out before a doctor can diagnose the condition as pulmonary fibrosis. A chest x-ray can reveal whiteness in the lungs consistent with fibrous tissue. CT scans can show fibrosis as can pulmonary function studies. Oximetry can show the amount of oxygen in the tissues, which may be low in pulmonary fibrosis. An exercise stress test can show changes in breathing with exercise.
To be sure that the problem is pulmonary fibrosis, the doctor can use a bronchoscope to do a transbronchial biopsy. This takes a tiny portion of the lung, which is then evaluated under the microscope for fibrosis. A broncho-alveolar lavage puts water into the lungs and takes the water back out, looking at the cells under the microscope that are gotten from the lavage. Surgery can be done that takes an open lung biopsy of the lung and can get a larger sample than either of the two prior procedures. It is sometimes used via a video assisted thorascopic surgery.
Treatment of pulmonary fibrosis includes taking steroids to block the inflammation that causes the fibrosis. Other drugs that suppress the immune system can be used, including cyclosporine and methotrexate. A newer drug, pirfenidone, has been found to reduce the damage to the lungs in the disease. There are serious side effects to taking these medications so they must be used carefully and at the lowest possible dosage.
As a last approach, doctors can consider doing a lung transplant to help improve the lung function. You cannot smoke if you want to be a candidate for lung transplant. There are a lot of medications you must take after the lung transplant to reduce the risk of transplant rejection.