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Idiopathic pulmonary fibrosis or just pulmonary fibrosis is a disease involving scar tissue or thickening of the lung tissue with no known cause. While no one knows the exact cause of the disease or why some people get it and others don't, the lungs become stiff and scarred so that it is difficult to breathe. The condition can come on gradually over a person's lifetime or can become suddenly very severe over a period of a few months or a few years. The condition is considered idiopathic because no one knows the cause of the disease. What is known is that it most commonly affects individuals between the ages of 50 and 70 years.

The major symptoms of pulmonary fibrosis include a dry cough, chest pain which is intermittent, reduced tolerance for physical activity and shortness of breath when active. The individual becomes a pulmonary cripple, unable to do things because of increasing shortness of breath with any activity. In end stage disease, there is shortness of breath all the time. The cause of these symptoms is the fibrous changes in the lungs which make excursion of the lungs a difficult thing to accomplish, even at rest.

The doctor can do various tests to determine if you have pulmonary fibrosis. The history you give the doctor is important, including exposure to asbestos or to other pulmonary irritants in the home or workplace. If you've been a smoker, you may be at higher risk. There may be crackles in the lungs during a physical examination that are from fibrotic changes in the lungs. There may also be cyanosis of the skin, especially around the mouth and in the fingernails. There can be clubbing of the nails, which involves an abnormal tip of the finger seen in many different kinds of pulmonary disease. The fingernail is primarily involved and curves over the finger, suggestive of a club at the tip of the nails.

Tests can also be done to diagnose idiopathic pulmonary fibrosis. These include a chest x-ray or a chest CT scan or MRI scan, which will show fibrotic changes in the lungs. A bronchoscopy can be performed that can take a biopsy of the lung, which will definitively show fibrosis of the lungs. Oxygen levels of the body can be assessed to see if they are low. Pulmonary function studies tell the way the lungs breathe in and out. You can have tests for connective tissue diseases, such as scleroderma or rheumatoid arthritis. You can also have an open lung biopsy or needle biopsy of the lungs which can define the presence of the disease.

There is no obvious or known cause for pulmonary fibrosis. There are no medications which stop the progression of the disease and it tends to progress, regardless of treatment. Inflammation can be controlled using corticosteroids and cytotoxic drugs. Oxygen is used to improve the blood oxygen levels. For some people, they can join a clinical trial to see if a drug is effective against the disease. In fact, they have nothing to lose when it comes to treating their disease. Lung rehabilitation will improve the function of the lungs using exercises to keep the exercise ability up to a decent level. A lung transplant is a last ditch effort to try and save the patient's life. The truth is that the disease tends to worsen regardless of the type of treatment used.

The prognosis of the disease is poor. Patients eventually die from this disease over time. It can take just a few months or several decades for this to happen, depending on the disease process.

Complications of idiopathic pulmonary fibrosis include chronic low oxygen levels, pneumothorax (air in the lung space), cor pulmonale (high blood pressure of the lung vessels), polycythemia (too many red blood cells), respiratory failure and pulmonary hypertension.

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Medical Negligence Solicitors

Our personal injury solicitors operate a specialist medical negligence compensation service. Our Pulmonary Fibrosis solicitors deal with claims using a no win no fee arrangement which means that if you dont win then you dont pay them their professional costs. If you would like legal advice at no cost with no further obligation just complete the contact form or email our lawyers offices or use the helpline and a Pulmonary Fibrosis solicitor will review your medical negligence compensation claim and phone you immediately.

HELPLINE: ☎ 1800 633 634

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