Pulmonary fibrosis?

Question

information on out come of illness and diagnosis and treatment

Answer 1

Best Answer - Chosen by Voters

Pulmonary Fibrosis
What is pulmonary fibrosis?

"Fibrosis" is a term used to refer to scarring, so pulmonary fibrosis means scarring throughout the lungs. Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes (sarcoidosis, Wegener’s granulomatosis ), infections, environmental agents (asbestos, silica, exposure to certain gases), exposure to ionizing radiation (such as radiation therapy to treat tumors of the chest), chronic conditions (lupus, rheumatoid arthritis), and certain medications.

In a condition known as hypersensitivity pneumonitis, fibrosis of the lung can develop following a heightened immune reaction to inhaled organic dusts or occupational chemicals. This condition most often results from inhaling dust contaminated with bacterial, fungal, or animal products.

In some people, chronic pulmonary inflammation and fibrosis develop without an identifiable cause. Most of these people have a condition called idiopathic pulmonary fibrosis (IPF) that does not respond to medical therapy, while some of the other types of fibrosis, such as nonspecific interstitial pneumonitis (NSIP), may respond to immune suppressive therapy.

Synonyms (other names) for various types of pulmonary fibrosis that have been used in the past include chronic interstitial pneumonitis, Hamman-Rich Syndrome, and diffuse fibrosing alveolitis.

What are pulmonary fibrosis symptoms?

Symptoms of pulmonary fibrosis include shortness of breath, coughing and diminished exercise tolerance. The severity of symptoms and the progression (worsening) of symptoms over time can vary and are at least partially dependent upon the cause of the fibrosis.

How is pulmonary fibrosis diagnosed?

Pulmonary fibrosis is suggested by a history of progressive (worsening over time) shortness of breath with exertion. Sometimes, during examination of the lungs with a stethoscope, the doctor can hear crackling sounds in the chest. The chest x-ray may or may not be abnormal, but a special x-ray test called a high resolution CAT scan will frequently demonstrate abnormalities. Lung function testing is distinctly abnormal.

The diagnosis can be confirmed by lung biopsy. An open surgical biopsy, meaning that the chest wall must be surgically opened under general anesthesia to remove a portion of lung tissue, may be necessary to obtain enough tissue to make an accurate diagnosis. The removed tissue is examined microscopically by a pathologist to confirm the presence of fibrosis.

How is the pulmonary fibrosis treated?

The treatment options for idiopathic pulmonary fibrosis are very limited. There is no evidence that any medications can help this condition, since scarring is permanent once it has developed. Lung transplantation is the only therapeutic option available. At times, this diagnosis can be difficult to make even with tissue biopsy reviewed by pathologists with specific experience in this field. Research trials using different drugs that may reduce fibrous scarring are ongoing. Since some types of lung fibrosis can respond to corticosteroids (such as Prednisone) and/or other medications that suppress the body's immune system, these types of drugs are sometimes prescribed in an attempt to decrease the processes that lead to fibrosis.

The immune system is felt to play a central role in the development of many forms of pulmonary fibrosis. The goal of treatment with immune suppressive agents such as corticosteroids is to decrease lung inflammation and subsequent scarring. Responses to treatment are variable. Once scarring has developed, it is permanent. Those whose conditions improve with immune suppressive treatment probably do not have idiopathic pulmonary fibrosis.

The toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist experienced in this condition. The lung specialist will determine the need for treatment, the duration of treatment, and will monitor the response to therapy along with any side effects. Only a minority of patients respond to corticosteroids alone, so other immune-suppressing medications are used in addition to corticosteroids. These include gamma-interferon, cyclophosphamide, azathioprine, methotrexate, penicillamine, and cyclosporine. The anti-inflammatory medication colchicine has also been used with limited success. Ongoing trials are underway using newer drugs such as gamma interferon, mycophenolate mofetil (Cellcept), and pirfenidone.

Pulmonary fibrosis can cause decreased oxygen levels in the blood. A decrease in blood oxygen level (hypoxia) can lead to elevated pressure in the pulmonary artery (the vessel that carries blood from the heart to the lungs to receive oxygen), a condition known as pulmonary hypertension, which can in turn lead to failure of the right ventricle of the heart. Therefore, patients with pulmonary fibrosis are frequently treated with supplemental oxygen to prevent pulmonary hypertension.

There is also evidence that patients suffering from pulmonary fibrosis may be at increased risk for blood clots that travel to the lung (pulmonary emboli), and therefore anticoagulation (blood thinning) therapy may be indicated

For more see: http://www.medicinenet.com/pulmonary_fib…

Source(s):

Google Search
http://www.medicinenet.com/pulmonary_fib…

Answer 2

If one of the known causes of pulmonary fibrosis exists, then treatment of that underlying disease or removal of the patient from the environment causing the disease can be effective.


A chest x-ray may give some clues. Pulmonary function tests, which reveal the efficiency of a patient's breathing mechanism, may give other indications.
Bronchoalveolar lavage (a test which permits removal and examination of cells in the lower respiratory tract) is sometimes used for diagnosis. Another diagnostic technique involves injection with gallium 67, a substance which is absorbed by inflamed areas that can be seen later on sensitized film.
At some point, a lung biopsy may be performed so that cells in and around the alveoli can be examined.


Many times the disease is mild with few symptoms and does not progress significantly with the years. In other cases, when pulmonary fibrosis is due to some other underlying disease such as rheumatoid arthritis, progression of the lung condition may reflect progression of the underlying diseases. Very rarely pulmonary fibrosis has a sudden onset and rapidly progresses to death from respiratory failure over a period of weeks. However, the usual course of pulmonary fibrosis, particularly idiopathic pulmonary fibrosis, is one of slowly progressive scarring of the lungs. The duration and speed of this process is variable. Some patients do not respond to therapy and have a slow deterioration over months to years, eventually ending in death when lungs can no longer function adequately. In other cases, patients respond to therapy.

Source(s):

http://www.lung.ca/diseases-maladies/a-z…

Answer 3

I was diagnosed with interstitial pulmonary fibrosis last year. I chose to deal with it from a nutritional way. My disease is now in remission ! Go to my web site and see what I used. naturesmagik.us you will even see a Drs report showing my results.

Source(s):

My story on naturesmagik.us

Answer 4

CFA in the UK. cryptogenic fibrosing alveolitis.

If you have just had a CT scan then it is not conclusive. My CT scan had the ground glass features that are an excellent indicator of the disease. The next step is a lung biopsy which luckily I avoided. I was down graded to NSIP.

Some of the links are technical but these are the most useful I found

Source(s):

http://home.netcarrier.com/~rstevens/

http://www.britishlungfoundation.org/fib…

http://www.emedicine.com/med/topic1960.h…

Answer 5

What is Pulmonary Fibrosis?

Pulmonary Fibrosis involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissue’s ability to transfer oxygen into the bloodstream.

What are the symptoms?

Shortness of breath, particularly with exertion

Chronic dry, hacking cough

Fatigue and weakness

Discomfort in the chest

Loss of appetite

Rapid weight loss

What is the prevalence of Pulmonary Fibrosis?

There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually. This is the same as die from Breast Cancer. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group.

What are the causes?

Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following:

Inhaled environmental and occupational pollutants

Cigarette smoking

Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis

Certain medications

Therapeutic radiation

How is it treated?

There are currently no effective treatments or a cure for Pulmonary Fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress.

Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly.

There are a number of new trials testing drugs to treat Pulmonary Fibrosis.

Answer 6

www.PulmonaryFibrosis.org; www.nlm.nih.gov/medlineplus/pulmonaryfib…
www.mayoclinic.com/health/pulmonary-fi…

Source(s):

respiratory professional