Pulmonary Fibrosis

Pulmonary Fibrosis General Information

Pulmonary Fibrosis is one of the most common types of interstitial lung diseases, along with desquamative interstitial pneumonia, lymphoid interstitial pneumonia, acute interstitial pneumonia, cryptogenic organizing pneumonia, nonspecific interstitial pneumonia and respiratory bronchiolitis (which is an associated interstitial lung disease). The disease has a higher frequency of occurrence in patients between 50 and 70 years of age, and it is thought to be caused by such factors as cigarette smoking, inhalation of pollutants, whether environmental or occupational, several medications, radiation (therapeutic radiation included) or various medical affections, such as rheumatoid arthritis, Scleroderma, Sarcoidosis or Lupus.
 
Pulmonary Fibrosis causes the lungs to stiffen and scar in the affected patient, which causes pronounced breathing problems as fibrotic tissue progressively replaces the air sacs. At the locations where the scars form, the tissue thickens and permanently loses its ability to bind oxygen into the blood cells, effectively destroying the functionality of the cells. In some patients, the condition has been noted to aggravate relatively quick, within a few months, while in others the disease may take years to evolve. It has not yet been discovered what determines the speed at which the disease evolves, but some recent studies show that smoking may play a part in it as a much higher percentage of smokers affected by this condition experienced a faster evolution of the disease than non-smoking patients.

Pulmonary Fibrosis Symptoms

In the case of Pulmonary Fibrosis, the nature and severity of the symptoms may depend largely on the stage the disease has advanced to and on the extent of the damage inflicted on the patient’s lungs. There may be symptoms that belong to other medical conditions, which may appear as complications as the medical condition of the patient worsens; failure of the right side of the heart or lung infections have been noted in the advanced stages of the affection.

Among the first symptoms that a patient will experience is typically shortness of breath. This is manifested at first on exertion, but with time it may also extend to the periods of rest. Soon after that, a chronic, dry and hacking cough may set in. In this stage weakness and fatigue are often encountered, even after very short periods of activity. In some cases, weight loss may also appear. This stage may last for 6 months but in most cases it spans over several years, during which the symptoms of Pulmonary Fibrosis worsen gradually.

During this time, further damage is inflicted on the patient’s lungs causing a steady drop in the amount of oxygen that is being processed and transferred in the blood. The skin of the patient who is suffering from Pulmonary Fibrosis may develop a bluish color, and the tips of the fingers may start thickening and taking a club-like shape. The right ventricle of the heart starts enlarging under the strain, which in time may lead to failure of the right side of the heart. Stethoscope examination of the patient’s breathing sounds will reveal cracking sounds in the patient’s chest. The patient may experience chest pains or discomfort and generally he presents an almost complete loss of appetite.

Pulmonary Fibrosis Treatment

Several tests may be performed in order to diagnose the Pulmonary Fibrosis. Chest x-rays may reveal a netlike pattern of whitish lines throughout the affected areas of the lungs, generally in their lower parts. CT scans may also reveal these patterns, and again the density of the lines is generally higher in the lower parts of the lungs. By performing a series of pulmonary function tests, the examining health care professional may be able to determine that the lung’s capacity has been reduced, thus reducing the amount of air that can enter the lungs at any moment. Blood sample analysis may reveal a low level of oxygen in the blood of the patient. Also, the patient may present abnormal breath sounds, much like the sound produced by Velcro when it is pulled apart.

Further testing may be done in order to confirm the diagnosis. Lung biopsy performed through bronchoscopy may be employed in such cases to verify that the patient is indeed suffering from Pulmonary Fibrosis; in some situations, a large tissue sample must be removed surgically to allow for further studies, and in order to obtain this sample the specialized health care professional may use a thoracoscope. While blood testing may not provide sufficient information to determine the exact nature of the affection, such tests may be done in order to eliminate a number of other medical conditions that may share symptoms with Pulmonary Fibrosis, such as certain autoimmune disorders.

The general life expectancy of patients suffering from this condition is of 3 to 5 years, however in some cases the patients have lived for longer than that, up to a decade after the diagnosis of the disease. However, in a few other cases the state of the patient worsened rapidly and the patient died within a few months from the examination. To prevent such situations, it is advised that the patient is kept under medical observation, to monitor the evolution of the affection and if needed to provide emergency medical attention.

Unfortunately, at this time there is no known cure for Pulmonary Fibrosis. The treatments that may be applied are generally aimed at relieving the symptoms of the disease and improving the quality of life for the patient. In cases where lung scarring is not extensive, the examining health care professional may prescribe corticosteroids or Azathioprine; another therapy which is commonly employed is to prescribe Prednisone in high doses for three months and gradually reduce the dosage over the following three months. Treatment may continue for up to another 6 months with very low doses of the same drug. Studies have also shown some results in extending the patient’s life expectancy by reducing lung fibrosis through treatment with Bosentan and Pirfenidone, however these therapies are still experimental and no clear results have been made public.

Rehabilitation techniques that are employed in order to help relieve symptoms and which may allow the patient to resume his or her normal life activities include pulmonary rehabilitation, oxygen therapy (this is aimed at improving the oxygen levels in the patient’s blood), drugs in order to counter the possibility of heart failure, and antibiotics to prevent or reduce infections. In some cases, lung transplant operations have been successful.