Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis General Information

Idiopathic Pulmonary Fibrosis, also known under the generic medical term of cryptogenic fibrosing alveolitis, is the most common type of idiopathic interstitial pneumonia along with desquamative interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, lymphoid interstitial pneumonia, respiratory bronchiolitis (which is an associated interstitial lung disease) and nonspecific interstitial pneumonia. At this time it still has not yet been established what exactly causes this affection – the term “idiopathic” literally means “with no known cause”. It is generally believed that it may result from the inflammatory reaction to a certain substance, but testing is still being performed o discover the exact compound causing this reaction in the organism. The disease has a higher frequency of occurrence in patients between 50 and 70 years of age.

Idiopathic Pulmonary Fibrosis causes the patient’s lungs to stiffen and scar, which causes the patient to begin experiencing more and more 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 cells from a functional point of view. In some patients, the state of the condition has been reported to aggravate relatively quick, within a few months, while in others the disease takes years to progress. It has not been ascertained what determines the speed at which the illness evolves, but some recent studies seem to show that smoking may play a part in it as a much higher percentage of the smokers affected by this condition experienced a faster evolution of the disease than the non-smoking patients.

Idiopathic Pulmonary Fibrosis Symptoms

In the case of Idiopathic Pulmonary Fibrosis, the nature and severity of the symptoms largely depend on the stage the disease has reached and on the extent of the damage inflicted on the lungs. There may be complications of the disease that also add symptoms as the condition of the patient worsens; failure of the right side of the heart or lung infections have been noted in patients in the advanced stages of the affection.

The first symptom that the patient will experience is a shortness of breath that is manifested on exertion, but with time it will also extend to the periods of rest as well. Soon after that, the patient will be affected by a chronic cough, hacking and dry throat. In this stage the patient begins feeling weak and fatigued even after very short periods of activity. In some cases, weight loss has also been observed. This stage may last for 6 months to several years, over which the Idiopathic Pulmonary Fibrosis symptoms gradually worsen.

During this time, the amount of damage inflicted on the lungs constantly increases causing a steady drop in the amount of oxygen that is being transferred in the blood. The skin of the patient who is suffering from Idiopathic Pulmonary Fibrosis may gradually turn bluish, and the tips of the fingers start thickening or taking a club-like shape. The right ventricle starts enlarging under the strain, in time leading to failure of the right side of the heart. Cracking sounds become audible in the patient’s chest through a stethoscope. At this point, the patient may experience chest pain or discomfort and an almost complete loss of appetite.

Idiopathic Pulmonary Fibrosis Treatment

There are several tests that may be done to diagnose the Idiopathic Pulmonary Fibrosis in a patient. Chest x-rays will reveal a net like pattern of white lines throughout the affected areas of the lungs, especially in the lower parts. CT scans will also show such patterns, and again the density of the lines will be higher in the lower parts of the lungs. By performing a series of pulmonary function tests, the examining health care specialist will be able to determine that the lung’s capacity is reduced, by that referring to the amount of air that can enter the lungs at any moment. Blood sample analysis will reveal a low level of oxygen in the blood of the affected 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 to confirm the diagnosis. Lung biopsy through bronchoscopy may be employed to verify that the patient is indeed suffering from Idiopathic Pulmonary Fibrosis; in some cases, a large tissue sample must be removed surgically for further studies, and in order to obtain it the specialized health care professional may employ the use of a thoracoscope. While blood testing may not prove conclusive in determining the exact nature of the affection, they may be done in order to rule out a number of other medical conditions that may share symptoms with Idiopathic Pulmonary Fibrosis, such as certain autoimmune disorders.

Once the diagnosis has been verified and it has been clearly determined that the patient is indeed suffering from this condition, the life expectancy is typically of 3 to 5 years. Some cases have been noted where the patients have lived for longer than that timeframe, up to a decade after the diagnosis was placed, however there are also a few cases where the state of the patient worsens rapidly and the patient dies within a few months. It is advised that the patient is kept under medical observation, to monitor the evolution of the affection.

Unfortunately, at this time there is no known cure for Idiopathic Pulmonary Fibrosis. The treatments that may be applied are aimed at relieving the symptoms that the patient may experience and improving the general health condition of the patient. In cases where lung scarring is not extensive, the examining health care specialist may prescribe corticosteroid and Azathioprine; a common therapy is prescribing Prednisone in high doses for three months and gradually reducing the dosage over the following three months. Treatment may continue with another 6 months of very low doses of the same drug. Studies have also shown some results in extending the patient’s survival timeframe by reducing lung fibrosis through treatment with Bosentan and Pirfenidone, however these are still experimental therapies and no clear results have been made public.

Rehabilitation techniques that help relieve symptoms and may allow the patient to resume the normal life activities include pulmonary rehabilitation, oxygen therapy that is aimed to improve the blood oxygen levels, drugs to counter the possibility of heart failure as well as antibiotics treatment to prevent or reduce infections. In some cases, attempts at lung transplant have been successful.